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Pathology > 4 - Diseases Of The Immune System > Flashcards

4 - Diseases Of The Immune System Flashcards

1
Q

Refers to the body’s protection against infection

A

Immunity

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2
Q

Composed of innate and adaptive components
Capable of causing tissue injury
May become exaggerated or depressed

A

Immune System

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3
Q

Natural, present at birth, cells are always present, non-adapative

A

Innate immune system

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4
Q

Characterized by inflammation, antiviral defense and sending danger signals

A

Innate immune system

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5
Q

Major reaction of innate immune system

A

Inflammation

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6
Q

Developed by exposure to pathogens, specific, normally silent

A

Adapative

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7
Q

Two components of adaptive immune system

A

Humoral

Cellular

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8
Q

Primary cells in humoral response

A

B cells

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9
Q

Primary cells in cellular response

A

T cells

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10
Q

Three broad categories of the diseases of the immune system

A
  1. Hypersensitivity reactions
  2. Autoimmune disease
  3. Immunologic deficiency syndromes
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11
Q

A disease characterized by abnormal proteins from IgG that accumulates forming insoluble structures and exert obstructive and compressive effects

A

Amyloidosis

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12
Q

A category of immune system disease that may be

1) elicited by both endogenous and exogenous substances
2) associated with particular susceptible genes
3) caused by imbalance between effector and control mechanisms

A

Hypersensitivity reactions

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13
Q

Exaggerated immune system

A

Hypersensitivity reactions

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14
Q

Depressed immune system

A

Immunologic deficiency syndromes

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15
Q

Self against self

A

Autoimmune diseases

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16
Q

Four types of hypersensitivity reactions

A

Type I: Immediate hypersensitivity
Type II: Antibody-mediated hypersensitivity
Type III: Immune complex mediated hypersensitivity
Type IV: T-cell mediated hypersensitivity

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17
Q

Type of hypersensitivity reactions characterized by rapidly developing immunologic reaction occurring within minutes after the combination of an antigen with antibody bund to mast cells in individuals previously sensitized to the antigen

A

Type I: Immediate hypersensitivity

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18
Q

Type I: Immediate hypersensitivity reaction is mediated by this antibody

A

IgE

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19
Q

Mechanism of type I: Immediate hypersensitivity reactions

A

Ag + IgE bound to mast cells or basophils ➡️ immediate release of vasoactive amines and other mediators from mast cells and recruitment of inflammatory cells

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20
Q

Mechanism of Type I: Immediate hypersensitivity reactions on re-exposure

A

Allergen binds and crosslink with IgE on mast cells results in:

  1. Release (degranulation) of preformed vesicles containing primary mediators
  2. De novo synthesis and release of secondary mediators
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21
Q

Primary mediators of Type I: Immediate hypersensitivity reactions

A

Histamine, proteases, chemotactic factors

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22
Q

Main mediator responsible for signs and symptoms of Type I: Immediate hypersensitivity reactions

A

Histamine

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23
Q

Secondary mediators of Type I: Immediate hypersensitivity reactions

A

Leukotrienes B4, C4, D4 and prostaglandin D2

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24
Q

Two phases of Type I: Immediate hypersensitivity reactions

A
  1. Initial (rapid) response: 5-30mins

2. Second (delayed) phase: 2-24hrs

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25
Q

Mediators of initial response phase

A

Biogenic amines

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26
Q

Effect of biogenic amines

A

Bronchial smooth muscle contraction
Increased vascular permeability and dilatation
Increase mucous gland secretion

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27
Q

Mediators of second phase

A

Lipid mediators: LTB4, C4, D4 & E4, PGD2 and PAF

Cytokine mediators: TNF-alpha, IL-1,3,4,5,6, GM-CSF, chemokines

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28
Q

Effects of Leukotriene C4 and D4

A

Bronchial smooth muscle contraction

Increase permeability

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29
Q

Effects of prostaglandin D2

A

Bronchospasm

Increase mucous secretion

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30
Q

Caused tissue damage by releasing major basic protein and cationic proteins

A

Eosinophils

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31
Q

True or False.

Major basic protein and eosinophil cationic proteins are both toxic to epithelial cells.

A

True

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32
Q

Effects of IL-3,4,5,6 and GM-CSF

A

Promote activation of IgE and recruitment of eosinophils

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33
Q

True or False.

Mediators take action on antigen and cause the clinical presentation of allergy

A

True

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34
Q

Mediator that induces class switching of the lymphocytes to an IgE producing B cell

A

IL-4

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35
Q

Mediator that activated eosinophils and recruits them to site of infection

A

IL-5

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36
Q

Receptors where IgE crosslinks with mast cells

A

FCepsilonR1

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37
Q

Two subtypes of Type I: Immediate hypersensitivity reactions

A
  1. Local immmediate hypersensitivity

2. Systemic anaphylaxis

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38
Q

Subtype of Type I: Immediate hypersensitivity that affects a specific organ, exemplified by atopic allergies, and have hereditary predisposition

A

Local immediate hypersensitivity reactions

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39
Q

True or False.
In local immediate hypersensitivity reactions, affected individuals tend to develop local type I responses to common allergens due to increase IgE and IL-4 producing TH2 cells.

A

True

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40
Q

Symptoms of local immediate hypersensitivity reactions

A

Utricaria, allergic rhinitis, asthma and nasal catarrh

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41
Q

Subtype of Type I: Immediate hypersensitivity that affects multiple organs, follows a parenteral or oral administration of an allergen (e.g. penicillin, peanuts, toxins, shellfish) and is more severe or may be fatal

A

Systemic anaphylaxis

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42
Q

Symptoms of systemic anaphylaxis

A

Pruritus, utricaria, erythema within minutes after exposure ➡️ bronchoconstriction, laryngeal edema, obstruction, hypotensive shock ➡️ death

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43
Q

Type of hypersensitivity reaction that is mediated by antibodies against intrinsic antigens or extrinsic agents adsorbed on cell surfaces or ECM

A

Type II: Antibody-mediated hypersensitivity reactions

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44
Q

Main antibodies involved in Type II: Antibody-mediated hypersensitivity reactions

A

IgG and IgM

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45
Q

Mechanism of Type II: Antibody-mediated hypersensitivity reactions

A

IgG or IgM + Ag bound to cell surface or ECM ➡️ phagocytosis and lysis ➡️ tissue injury due to inflammation

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46
Q

Three major pathways of Type II: Antibody-mediated hypersensitivity reactions

A

IIa. Opsonization, complement- and Fc-mediated phagocytosis
IIb. Complement- and Fc-receptor-mediated inflammation
IIc. Antibody-mediated cellular dysfunction

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47
Q

Components of the complement system that act as opsonins

A

C3b and C5b

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48
Q

True or False.
Complement activation causes neutrophils and macrophages to attack Ag which release ROS and substances that induce inflammation

A

True

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49
Q

Autoimmune hemolytic anemia
Target antigen: ________________
Mechanism: __________________

A

Target antigen: RBC membrane proteins

Mechanism: IIa opsonization and phagocytosis of erythrocytes

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50
Q

Autoimmune thrombocytopenia purpura
Target antigen: ________________
Mechanism: __________________

A

Target antigen: Platelet membrane proteins (GpIIb:IIa or GpIb/IX)
Mechanism: IIa opsonization and phagocytosis of platelets

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51
Q

Goodpasture syndrome
Target antigen: ________________
Mechanism: __________________

A

Target antigen: Noncollagenous proteins in basement membrane of alveoli and glomeruli
Mechanism: IIb complement and Fc receptor mediated inflammation

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52
Q

Myasthenia gravis
Target antigen: ________________
Mechanism: __________________

A

Target antigen: Acetylcholine receptor

Mechanism: IIc Ab inhibits Ach binding; down-regulates receptor

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53
Q 
Graves disease (hyperthyroidism)
Target antigen: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Mechanism: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
A

Target antigen: TSH receptor

Mechanism: IIc Ab-mediated stimulation of TSH receptor

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54
Q

Insulin resistance DM
Target antigen: ________________
Mechanism: __________________

A

Target antigen: Insulin receptor

Mechanism: IIc Ab inhibit binding of insulin

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55
Q

Acute Rheumatic Fever
Target antigen: ________________
Mechanism: __________________

A

Target antigen: Streptococcal cell wall Ag (exogenous)
Mechanism: Ab mistakes myocardial Ag as foreign; IIb Ab cross-reacts with host myocardial Ag ➡️ macrophage activation/inflammation

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56
Q

Mechanism of Type III: Immune-complex mediated hypersensitivity

A

Ag+Ab ➡️ Ag-Ab complexes in circulation will be deposited in various organs ➡️ activate complement (classical pathway) ➡️ attract neutrophils and macrophages ➡️ release lysosomal enzymes (elastases, collagenases) ➡️ vasculitis, glomerulonephritis, arthritis, rash, etc.

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57
Q

Two subtypes of Type III: Immune-complex mediated hypersensitivity

A

Local

Systemic

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58
Q

Common deposition of immune-complexes

A

Kidneys, joints, blood vessels and skin

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59
Q

True or False.

Immune complexes are hard to eliminate and may bond in the circulation or at site of deposition.

A

True

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60
Q

In this subtype of Type III: Immune-complex mediated hypersensitivity, circulating immune complexes are systemically deposited, particularly in organs that filter blood at high pressures

A

Systemic immune complex disease

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61
Q

Examples of systemic immune complex disease

A

Acute serum sickness

SLE

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62
Q

Clinical presentation of acute serum sickness

A

Arthritis, skin rash, fever

4-5 days after admin of serum but may appear more rapidly with repeated injection of the serum

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63
Q

Phases of the pathogenesis of systemic immune complex disease

A

Phase I: Formation of Ag-Ab complexes in circulation
Phase II: Deposition of immune complexes in various tissues
Phase III: Inflammatory reaction at the sites of immune complex deposition and clinical features appear due to tissue injury 10 days after exposure

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64
Q

True or False.

During active phase, C3 levels are decrease because they are used.

A

True

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65
Q

Characteristics of local immune complex disease

A

Affects a particularly
Arthus reaction
Large immune complexes precipitate in the vessel walls causing disruption of blood flow and inflammation
Visible edema with severe hemorrhage followed occassionally by ulceration of the skin

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66
Q

Morphologic manifestations of local immune complex disease

A

Acute vasculitis with fibrinoid necrosis of the vessel wall

Intense neutrophilic infiltration

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67
Q

Disease examples of Ab-mediated hypersensitivity

A 
Autoimmune hemolytic anemia
Autoimmune thrombocytopenia purpura
Goodpasture syndrome
Myasthenia gravis
Graves disease
Insulin resistance DM
Acute rheumatic fever
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68
Q

Disease examples of immune-complex mediated hypersensitivity

A 
SLE
Polyarteritis nodosa
Poststreptococcal glomerulonephritis
Acute glomerulonephritis
Arthus reaction
Serum sickness
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69
Q

Localized are of tissue necrosis resulting from acute immune complex vasculitis, usually elicited in the skin

A

Arthus reaction

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70
Q

SLE
Antigen involved: _______________
Manifestations: _______________

A

Antigen involved: DNA, nucleoproteins, others

Manifestations: Nephritis, arthritis, vasculitis, skin lesions

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71
Q

Polyarteritis nodosa
Antigen involved: _______________
Manifestations: _______________

A

Antigen involved: Hep B surface antigen

Manifestations: Vasculitis

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72
Q

Poststreptococcal glomerulonephritis
Antigen involved: _______________
Manifestations: _______________

A

Antigen involved: Streptococcal cell wall Ags, may be planted in GBM
Manifestations: Nephritis

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73
Q

Acute glomerulonephritis
Antigen involved: _______________
Manifestations: _______________

A

Antigen involved: Bacterial, parasite, tumor Ag

Manifestations: Nephritis

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74
Q

Arthus reaction
Antigen involved: _______________
Manifestations: _______________

A

Antigen involved: Various foreign proteins

Manifestations: Cutaneous vasculitis

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75
Q

Serum sickness
Antigen involved: _______________
Manifestations: _______________

A

Antigen involved: Various proteins (e.g. foreign serum)

Manifestations: Arthritis vasculitis, nephritis

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76
Q

This type of hypersensitivity reaction is initiated by Ag-activated (sensitized) lymphocytes

A

Type IV: T-cell mediated hypersensitivity

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77
Q

Two subtypes of Type IV: T-cell mediated hypersensitivity

A

Delayed type hypersensitivity

T-cell mediated cytotoxicity

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78
Q

This subtype of Type IV: T-cell mediated hypersensitivity reaction is a principal pattern of response to TB, fungi, protozoa, parasite, contact skin sensitivity and allograft rejection

A

Delayed type hypersensitivity

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79
Q

Cytokines release during delayed type hypersensitivity

A

IFN-gamma
IL2
TNF and Lymphotoxin

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80
Q

Cytokine responsible for activation of macrophages

A

IFN-gamma

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81
Q

Cytokine responsible for the proliferation T cells

A

IL2

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82
Q

Cytokines that are responsible for the extravasation of lymphocytes & monocytes and granuloma formation

A

TNF and Lymphotoxins

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83
Q

The type of hypersensitivity reaction whose inflammatory response is the most destructive

A

Type IV: T-cell mediated hypersensitivity

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84
Q

True or False.

CD4+ T cells and macrophages are always partners.

A

True

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85
Q

True or False.

Sometimes, CD8+ T cells will play a role in producing cytokines to aid CD4+ T cells.

A

True

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86
Q

Major T-cell in delayed type hypersensitivity reactions

A

CD4+ T cells

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87
Q

Major T cell in T-cell mediated cytotoxicity

A

CD8+ T cell

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88
Q

Morphology of delayed type hypersensitivity reactions

A

Perivascular cuffing
Perivascular infiltrate replaced by macrophages (after 2-3 weeks) with epithelioid cells
Granuloma formation

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89
Q

Mechanism of delayed type hypersensitivity reaction

A

Ag ➡️ Proliferation and differentiation of CD4+ T cells (APCs produce IL-1,6,12,23 and IFN-gamma) ➡️ Differentiation from TH1 to TH17 subset ➡️ inflammation

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90
Q

Type IV: T-cell mediated hypersensitivity subtype that fights against virus, tumor cells and allograft rejection

A

T-cell mediated cytotoxicity

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91
Q

Mechanism of T-cell mediated cytotoxicity

A

1.

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92
Q

Protease that cleave and activate caspases

A

Granzyme

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93
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

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94
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
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95
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

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96
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

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97
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

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98
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

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99
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

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100
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

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101
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

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102
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

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103
Q

Cells involved in acute humoral rejection

A

B cells and Abs

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104
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

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105
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

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106
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

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107
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

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108
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

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109
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

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110
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

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111
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

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112
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

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113
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

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114
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

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115
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
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116
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

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117
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

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118
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

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119
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

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120
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

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121
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

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122
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

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123
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
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124
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
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125
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

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126
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
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127
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

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128
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
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129
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

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130
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

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131
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

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132
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

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133
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
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134
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

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135
Q

Most common cause of death in SLE

A

Renal failure

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136
Q

Second most common cause of death in SLE

A

Sepsis/infection

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137
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
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138
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
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139
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
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140
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

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141
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
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142
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

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143
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

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144
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

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145
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

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146
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

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147
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
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148
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

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149
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

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150
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

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151
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
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152
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

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153
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

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154
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

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155
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

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156
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

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157
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

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158
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

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159
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
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160
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

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161
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

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162
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

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163
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

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164
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

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165
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

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166
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
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167
Q

Calcium deposit in the skin

A

Calcinosis

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168
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

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169
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

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170
Q

Skin thickening of the fingers

A

Sclerodactyly

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171
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

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172
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

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173
Q

Two types of immunodeficiency

A

Primary

Secondary

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174
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

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175
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

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176
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

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177
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

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178
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
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179
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

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180
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

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181
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

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182
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

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183
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

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184
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

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185
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

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186
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

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187
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

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188
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

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189
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

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190
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

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191
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

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192
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

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193
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

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194
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

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195
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

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196
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

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197
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

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198
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

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199
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

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200
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

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201
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

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202
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

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203
Q

Promote survival and differentiation of B cells

A

BAFF

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204
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

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205
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

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206
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

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207
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

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208
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

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209
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

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210
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

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211
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

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212
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

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213
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

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214
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

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215
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

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216
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
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217
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
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218
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
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219
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

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220
Q

Two major target of HIV

A

Immune system and CNS

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221
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

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222
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

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223
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

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224
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

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225
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

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226
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

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227
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

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228
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

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229
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

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230
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

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231
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

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232
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

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233
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

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234
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

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235
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

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236
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

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237
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

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238
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

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239
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

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240
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

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241
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
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242
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

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243
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

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244
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

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245
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

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246
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
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247
Q

Protease that cleave and activate caspases

A

Granzyme

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248
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

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249
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
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250
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

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251
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

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252
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

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253
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

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254
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

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255
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

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256
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

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257
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

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258
Q

Cells involved in acute humoral rejection

A

B cells and Abs

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259
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

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260
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

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261
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

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262
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

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263
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

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264
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

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265
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

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266
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

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267
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

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268
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

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269
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

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270
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
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271
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

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272
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

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273
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

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274
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

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275
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

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276
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

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277
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

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278
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
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279
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
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280
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

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281
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
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282
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

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283
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
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284
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

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285
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

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286
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

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287
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

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288
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
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289
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

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290
Q

Most common cause of death in SLE

A

Renal failure

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291
Q

Second most common cause of death in SLE

A

Sepsis/infection

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292
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
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293
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
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294
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
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295
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

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296
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
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297
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

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298
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

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299
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

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300
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

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301
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

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302
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
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303
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

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304
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

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305
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

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306
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
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307
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

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308
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

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309
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

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310
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

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311
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

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312
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

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313
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

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314
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
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315
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

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316
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

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317
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

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318
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

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319
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

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320
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

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321
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
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322
Q

Calcium deposit in the skin

A

Calcinosis

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323
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

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324
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

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325
Q

Skin thickening of the fingers

A

Sclerodactyly

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326
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

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327
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

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328
Q

Two types of immunodeficiency

A

Primary

Secondary

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329
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

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330
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

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331
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

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332
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

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333
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
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334
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

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335
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

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336
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

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337
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

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338
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

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339
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

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340
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

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341
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

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342
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

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343
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

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344
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

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345
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

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346
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

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347
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

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348
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

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349
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

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350
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

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351
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

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352
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

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353
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

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354
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

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355
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

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356
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

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357
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

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358
Q

Promote survival and differentiation of B cells

A

BAFF

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359
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

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360
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

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361
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

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362
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

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363
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

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364
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

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365
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

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366
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

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367
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

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368
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

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369
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

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370
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

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371
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
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372
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
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373
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
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374
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

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375
Q

Two major target of HIV

A

Immune system and CNS

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376
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

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377
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

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378
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

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379
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

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380
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

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381
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

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382
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

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383
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

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384
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

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385
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

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386
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

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387
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

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388
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

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389
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

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390
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

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391
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

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392
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

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393
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

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394
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

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395
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

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396
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
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397
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

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398
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

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399
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

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400
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

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401
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
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402
Q

Protease that cleave and activate caspases

A

Granzyme

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403
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

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404
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
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405
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

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406
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

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407
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

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408
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

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409
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

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410
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

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411
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

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412
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

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413
Q

Cells involved in acute humoral rejection

A

B cells and Abs

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414
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

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415
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

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416
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

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417
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

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418
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

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419
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

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420
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

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421
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

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422
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

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423
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

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424
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

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425
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
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426
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

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427
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

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428
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

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429
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

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430
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

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431
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

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432
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

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433
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
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434
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
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435
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

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436
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
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437
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

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438
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
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439
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

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440
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

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441
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

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442
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

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443
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
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444
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

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445
Q

Most common cause of death in SLE

A

Renal failure

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446
Q

Second most common cause of death in SLE

A

Sepsis/infection

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447
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
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448
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
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449
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
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450
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

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451
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
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452
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

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453
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

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454
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

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455
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

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456
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

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457
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
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458
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

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459
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

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460
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

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461
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
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462
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

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463
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

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464
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

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465
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

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466
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

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467
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

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468
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

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469
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
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470
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

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471
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

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472
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

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473
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

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474
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

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475
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

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476
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
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477
Q

Calcium deposit in the skin

A

Calcinosis

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478
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

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479
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

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480
Q

Skin thickening of the fingers

A

Sclerodactyly

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481
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

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482
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

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483
Q

Two types of immunodeficiency

A

Primary

Secondary

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484
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

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485
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

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486
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

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487
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

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488
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
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489
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

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490
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

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491
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

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492
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

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493
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

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494
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

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495
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

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496
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

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497
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

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498
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

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499
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

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500
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

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501
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

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502
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

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503
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

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504
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

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505
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

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506
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

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507
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

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508
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

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509
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

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510
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

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511
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

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512
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

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513
Q

Promote survival and differentiation of B cells

A

BAFF

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514
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

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515
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

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516
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

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517
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

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518
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

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519
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

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520
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

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521
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

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522
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

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523
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

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524
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

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525
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

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526
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
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527
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
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528
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
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529
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

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530
Q

Two major target of HIV

A

Immune system and CNS

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531
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

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532
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

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533
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

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534
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

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535
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

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536
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

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537
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

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538
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

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539
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

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540
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

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541
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

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542
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

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543
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

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544
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

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545
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

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546
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

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547
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

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548
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

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549
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

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550
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

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551
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
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552
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

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553
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

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554
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

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555
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

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556
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
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557
Q

Protease that cleave and activate caspases

A

Granzyme

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558
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

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559
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
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560
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

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561
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

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562
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

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563
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

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564
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

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565
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

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566
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

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567
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

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568
Q

Cells involved in acute humoral rejection

A

B cells and Abs

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569
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

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570
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

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571
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

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572
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

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573
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

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574
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

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575
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

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576
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

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577
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

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578
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

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579
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

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580
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
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581
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

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582
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

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583
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

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584
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

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585
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

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586
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

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587
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

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588
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
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589
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
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590
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

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591
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
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592
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

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593
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
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594
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

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595
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

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596
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

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597
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

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598
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
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599
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

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600
Q

Most common cause of death in SLE

A

Renal failure

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601
Q

Second most common cause of death in SLE

A

Sepsis/infection

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602
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
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603
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
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604
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
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605
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

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606
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
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607
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

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608
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

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609
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

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610
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

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611
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

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612
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
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613
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

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614
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

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615
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

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616
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
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617
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

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618
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

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619
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

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620
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

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621
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

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622
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

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623
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

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624
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
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625
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

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626
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

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627
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

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628
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

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629
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

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630
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

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631
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
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632
Q

Calcium deposit in the skin

A

Calcinosis

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633
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

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634
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

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635
Q

Skin thickening of the fingers

A

Sclerodactyly

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636
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

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637
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

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638
Q

Two types of immunodeficiency

A

Primary

Secondary

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639
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

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640
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

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641
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

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642
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

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643
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
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644
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

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645
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

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646
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

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647
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

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648
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

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649
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

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650
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

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651
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

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652
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

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653
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

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654
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

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655
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

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656
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

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657
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

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658
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

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659
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

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660
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

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661
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

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662
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

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663
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

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664
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

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665
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

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666
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

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667
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

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668
Q

Promote survival and differentiation of B cells

A

BAFF

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669
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

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670
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

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671
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

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672
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

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673
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

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674
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

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675
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

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676
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

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677
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

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678
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

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679
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

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680
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

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681
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
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682
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
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683
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
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684
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

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685
Q

Two major target of HIV

A

Immune system and CNS

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686
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

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687
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

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688
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

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689
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

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690
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

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691
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

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692
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

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693
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

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694
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

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695
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

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696
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

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697
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

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698
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

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699
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

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700
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

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701
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

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702
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

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703
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

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704
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

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705
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

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706
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
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707
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

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708
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

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709
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

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710
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

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711
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
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712
Q

Protease that cleave and activate caspases

A

Granzyme

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713
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

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714
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
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715
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

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716
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

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717
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

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718
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

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719
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

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720
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

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721
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

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722
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

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723
Q

Cells involved in acute humoral rejection

A

B cells and Abs

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724
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

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725
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

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726
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

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727
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

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728
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

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729
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

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730
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

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731
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

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732
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

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733
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

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734
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

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735
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
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736
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

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737
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

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738
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

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739
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

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740
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

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741
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

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742
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

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743
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
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744
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
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745
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

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746
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
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747
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

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748
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
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749
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

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750
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

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751
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

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752
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

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753
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
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754
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

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755
Q

Most common cause of death in SLE

A

Renal failure

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756
Q

Second most common cause of death in SLE

A

Sepsis/infection

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757
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
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758
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
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759
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
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760
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

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761
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
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762
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

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763
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

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764
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

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765
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

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766
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

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767
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
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768
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

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769
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

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770
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

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771
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
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772
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

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773
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

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774
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

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775
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

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776
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

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777
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

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778
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

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779
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
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780
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

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781
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

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782
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

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783
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

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784
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

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785
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

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786
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
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787
Q

Calcium deposit in the skin

A

Calcinosis

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788
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

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789
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

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790
Q

Skin thickening of the fingers

A

Sclerodactyly

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791
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

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792
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

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793
Q

Two types of immunodeficiency

A

Primary

Secondary

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794
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

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795
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

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796
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

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797
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

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798
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
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799
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

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800
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

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801
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

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802
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

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803
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

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804
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

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805
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

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806
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

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807
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

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808
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

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809
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

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810
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

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811
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

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812
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

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813
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

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814
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

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815
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

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816
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

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817
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

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818
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

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819
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

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820
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

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821
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

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822
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

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823
Q

Promote survival and differentiation of B cells

A

BAFF

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824
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

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825
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

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826
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

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827
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

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828
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

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829
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

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830
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

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831
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

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832
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

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833
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

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834
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

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835
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

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836
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
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837
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
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838
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
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839
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

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840
Q

Two major target of HIV

A

Immune system and CNS

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841
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

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842
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

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843
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

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844
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

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845
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

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846
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

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847
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

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848
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

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849
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

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850
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

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851
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

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852
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

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853
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

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854
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

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855
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

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856
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

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857
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

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858
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

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859
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

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860
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

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861
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
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862
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

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863
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

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864
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

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865
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

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866
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
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867
Q

Protease that cleave and activate caspases

A

Granzyme

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868
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

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869
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
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870
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

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871
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

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872
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

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873
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

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874
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

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875
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

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876
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

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877
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

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878
Q

Cells involved in acute humoral rejection

A

B cells and Abs

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879
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

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880
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

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881
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

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882
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

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883
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

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884
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

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885
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

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886
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

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887
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

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888
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

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889
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

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890
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
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891
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

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892
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

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893
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

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894
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

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895
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

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896
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

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897
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

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898
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
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899
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
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900
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

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901
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
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902
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

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903
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
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904
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

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905
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

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906
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

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907
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

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908
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
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909
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

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910
Q

Most common cause of death in SLE

A

Renal failure

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911
Q

Second most common cause of death in SLE

A

Sepsis/infection

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912
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
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913
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
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914
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
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915
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

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916
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
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917
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

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918
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

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919
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

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920
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

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921
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

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922
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
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923
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

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924
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

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925
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

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926
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
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927
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

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928
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

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929
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

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930
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

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931
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

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932
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

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933
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

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934
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
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935
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

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936
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

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937
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

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938
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

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939
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

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940
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

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941
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
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942
Q

Calcium deposit in the skin

A

Calcinosis

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943
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

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944
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

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945
Q

Skin thickening of the fingers

A

Sclerodactyly

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946
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

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947
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

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948
Q

Two types of immunodeficiency

A

Primary

Secondary

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949
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

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950
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

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951
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

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952
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

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953
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
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954
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

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955
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

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956
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

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957
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

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958
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

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959
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

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960
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

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961
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

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962
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

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963
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

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964
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

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965
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

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966
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

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967
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

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968
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

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969
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

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970
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

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971
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

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972
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

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973
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

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974
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

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975
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

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976
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

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977
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

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978
Q

Promote survival and differentiation of B cells

A

BAFF

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979
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

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980
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

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981
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

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982
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

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983
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

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984
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

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985
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

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986
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

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987
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

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988
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

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989
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

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990
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

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991
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
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992
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
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993
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
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994
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

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995
Q

Two major target of HIV

A

Immune system and CNS

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996
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

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997
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

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998
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

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999
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

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1000
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

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1001
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

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1002
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

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1003
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

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1004
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

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1005
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

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1006
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

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1007
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

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1008
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

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1009
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

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1010
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

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1011
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

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1012
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

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1013
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

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1014
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

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1015
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

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1016
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
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1017
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

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1018
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

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1019
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

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1020
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

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1021
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
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1022
Q

Protease that cleave and activate caspases

A

Granzyme

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1023
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

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1024
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
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1025
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

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1026
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

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1027
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

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1028
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

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1029
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

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1030
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

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1031
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

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1032
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

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1033
Q

Cells involved in acute humoral rejection

A

B cells and Abs

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1034
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

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1035
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

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1036
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

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1037
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

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1038
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

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1039
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

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1040
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

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1041
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

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1042
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

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1043
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

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1044
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

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1045
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
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1046
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

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1047
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

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1048
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

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1049
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

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1050
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

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1051
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

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1052
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

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1053
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
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1054
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
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1055
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

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1056
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
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1057
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

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1058
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
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1059
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

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1060
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

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1061
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

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1062
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

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1063
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
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1064
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

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1065
Q

Most common cause of death in SLE

A

Renal failure

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1066
Q

Second most common cause of death in SLE

A

Sepsis/infection

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1067
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
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1068
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
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1069
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
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1070
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

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1071
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
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1072
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

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1073
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

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1074
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

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1075
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

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1076
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

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1077
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
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1078
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

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1079
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

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1080
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

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1081
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
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1082
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

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1083
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

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1084
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

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1085
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

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1086
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

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1087
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

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1088
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

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1089
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
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1090
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

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1091
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

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1092
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

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1093
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

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1094
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

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1095
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

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1096
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
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1097
Q

Calcium deposit in the skin

A

Calcinosis

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1098
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

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1099
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

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1100
Q

Skin thickening of the fingers

A

Sclerodactyly

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1101
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

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1102
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

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1103
Q

Two types of immunodeficiency

A

Primary

Secondary

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1104
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

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1105
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

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1106
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

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1107
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

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1108
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
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1109
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

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1110
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

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1111
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

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1112
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

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1113
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

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1114
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

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1115
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

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1116
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

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1117
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

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1118
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

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1119
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

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1120
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

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1121
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

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1122
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

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1123
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

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1124
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

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1125
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

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1126
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

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1127
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

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1128
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

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1129
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

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1130
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

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1131
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

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1132
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

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1133
Q

Promote survival and differentiation of B cells

A

BAFF

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1134
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

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1135
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

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1136
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

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1137
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

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1138
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

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1139
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

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1140
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

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1141
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

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1142
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

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1143
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

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1144
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

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1145
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

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1146
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
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1147
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
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1148
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
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1149
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

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1150
Q

Two major target of HIV

A

Immune system and CNS

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1151
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

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1152
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

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1153
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

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1154
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

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1155
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

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1156
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

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1157
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

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1158
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

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1159
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

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1160
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

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1161
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

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1162
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

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1163
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

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1164
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

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1165
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

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1166
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

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1167
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

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1168
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

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1169
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

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1170
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

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1171
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
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1172
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

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1173
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

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1174
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

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1175
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

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1176
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
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1177
Q

Protease that cleave and activate caspases

A

Granzyme

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1178
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

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1179
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
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1180
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

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1181
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

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1182
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

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1183
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

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1184
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

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1185
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

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1186
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

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1187
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

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1188
Q

Cells involved in acute humoral rejection

A

B cells and Abs

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1189
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

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1190
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

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1191
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

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1192
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

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1193
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

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1194
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

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1195
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

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1196
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

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1197
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

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1198
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

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1199
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

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1200
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
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1201
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

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1202
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

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1203
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

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1204
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

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1205
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

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1206
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

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1207
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

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1208
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
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1209
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
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1210
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

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1211
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
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1212
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

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1213
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
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1214
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

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1215
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

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1216
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

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1217
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

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1218
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
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1219
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

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1220
Q

Most common cause of death in SLE

A

Renal failure

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1221
Q

Second most common cause of death in SLE

A

Sepsis/infection

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1222
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
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1223
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
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1224
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
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1225
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

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1226
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
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1227
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

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1228
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

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1229
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

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1230
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

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1231
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

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1232
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
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1233
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

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1234
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

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1235
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

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1236
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
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1237
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

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1238
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

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1239
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

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1240
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

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1241
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

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1242
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

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1243
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

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1244
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
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1245
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

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1246
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

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1247
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

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1248
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

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1249
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

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1250
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

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1251
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
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1252
Q

Calcium deposit in the skin

A

Calcinosis

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1253
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

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1254
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

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1255
Q

Skin thickening of the fingers

A

Sclerodactyly

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1256
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

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1257
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

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1258
Q

Two types of immunodeficiency

A

Primary

Secondary

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1259
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

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1260
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

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1261
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

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1262
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

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1263
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
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1264
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

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1265
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

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1266
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

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1267
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

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1268
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

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1269
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

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1270
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

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1271
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

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1272
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

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1273
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

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1274
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

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1275
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

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1276
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

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1277
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

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1278
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

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1279
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

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1280
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

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1281
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

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1282
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

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1283
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

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1284
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

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1285
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

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1286
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

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1287
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

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1288
Q

Promote survival and differentiation of B cells

A

BAFF

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1289
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

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1290
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

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1291
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

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1292
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

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1293
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

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1294
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

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1295
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

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1296
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

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1297
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

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1298
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

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1299
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

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1300
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

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1301
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
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1302
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
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1303
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
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1304
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

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1305
Q

Two major target of HIV

A

Immune system and CNS

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1306
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

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1307
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

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1308
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

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1309
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

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1310
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

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1311
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

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1312
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

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1313
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

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1314
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

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1315
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

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1316
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

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1317
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

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1318
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

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1319
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

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1320
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

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1321
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

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1322
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

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1323
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

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1324
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

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1325
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

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1326
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
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1327
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

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1328
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

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1329
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

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1330
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

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1331
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
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1332
Q

Protease that cleave and activate caspases

A

Granzyme

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1333
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

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1334
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
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1335
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

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1336
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

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1337
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

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1338
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

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1339
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

1340
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

1341
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

1342
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

1343
Q

Cells involved in acute humoral rejection

A

B cells and Abs

1344
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

1345
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

1346
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

1347
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

1348
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

1349
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

1350
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

1351
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

1352
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

1353
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

1354
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

1355
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
1356
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

1357
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

1358
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

1359
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

1360
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

1361
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

1362
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

1363
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
1364
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
1365
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

1366
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
1367
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

1368
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
1369
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

1370
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

1371
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

1372
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

1373
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
1374
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

1375
Q

Most common cause of death in SLE

A

Renal failure

1376
Q

Second most common cause of death in SLE

A

Sepsis/infection

1377
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
1378
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
1379
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
1380
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

1381
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
1382
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

1383
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

1384
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

1385
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

1386
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

1387
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
1388
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

1389
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

1390
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

1391
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
1392
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

1393
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

1394
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

1395
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

1396
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

1397
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

1398
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

1399
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
1400
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

1401
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

1402
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

1403
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

1404
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

1405
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

1406
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
1407
Q

Calcium deposit in the skin

A

Calcinosis

1408
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

1409
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

1410
Q

Skin thickening of the fingers

A

Sclerodactyly

1411
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

1412
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

1413
Q

Two types of immunodeficiency

A

Primary

Secondary

1414
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

1415
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

1416
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

1417
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

1418
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
1419
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

1420
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

1421
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

1422
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

1423
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

1424
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

1425
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

1426
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

1427
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

1428
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

1429
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

1430
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

1431
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

1432
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

1433
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

1434
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

1435
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

1436
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

1437
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

1438
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

1439
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

1440
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

1441
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

1442
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

1443
Q

Promote survival and differentiation of B cells

A

BAFF

1444
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

1445
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

1446
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

1447
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

1448
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

1449
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

1450
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

1451
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

1452
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

1453
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

1454
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

1455
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

1456
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
1457
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
1458
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
1459
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

1460
Q

Two major target of HIV

A

Immune system and CNS

1461
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

1462
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

1463
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

1464
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

1465
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

1466
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

1467
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

1468
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

1469
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

1470
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

1471
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

1472
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

1473
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

1474
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

1475
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

1476
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

1477
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

1478
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

1479
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

1480
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

1481
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
1482
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

1483
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

1484
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

1485
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

1486
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
1487
Q

Protease that cleave and activate caspases

A

Granzyme

1488
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

1489
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
1490
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

1491
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

1492
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

1493
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

1494
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

1495
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

1496
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

1497
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

1498
Q

Cells involved in acute humoral rejection

A

B cells and Abs

1499
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

1500
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

1501
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

1502
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

1503
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

1504
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

1505
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

1506
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

1507
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

1508
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

1509
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

1510
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
1511
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

1512
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

1513
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

1514
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

1515
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

1516
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

1517
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

1518
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
1519
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
1520
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

1521
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
1522
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

1523
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
1524
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

1525
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

1526
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

1527
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

1528
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
1529
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

1530
Q

Most common cause of death in SLE

A

Renal failure

1531
Q

Second most common cause of death in SLE

A

Sepsis/infection

1532
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
1533
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
1534
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
1535
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

1536
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
1537
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

1538
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

1539
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

1540
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

1541
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

1542
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
1543
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

1544
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

1545
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

1546
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
1547
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

1548
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

1549
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

1550
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

1551
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

1552
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

1553
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

1554
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
1555
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

1556
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

1557
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

1558
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

1559
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

1560
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

1561
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
1562
Q

Calcium deposit in the skin

A

Calcinosis

1563
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

1564
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

1565
Q

Skin thickening of the fingers

A

Sclerodactyly

1566
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

1567
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

1568
Q

Two types of immunodeficiency

A

Primary

Secondary

1569
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

1570
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

1571
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

1572
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

1573
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
1574
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

1575
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

1576
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

1577
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

1578
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

1579
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

1580
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

1581
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

1582
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

1583
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

1584
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

1585
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

1586
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

1587
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

1588
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

1589
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

1590
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

1591
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

1592
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

1593
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

1594
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

1595
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

1596
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

1597
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

1598
Q

Promote survival and differentiation of B cells

A

BAFF

1599
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

1600
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

1601
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

1602
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

1603
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

1604
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

1605
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

1606
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

1607
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

1608
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

1609
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

1610
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

1611
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
1612
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
1613
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
1614
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

1615
Q

Two major target of HIV

A

Immune system and CNS

1616
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

1617
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

1618
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

1619
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

1620
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

1621
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

1622
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

1623
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

1624
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

1625
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

1626
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

1627
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

1628
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

1629
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

1630
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

1631
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

1632
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

1633
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

1634
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

1635
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

1636
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
1637
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

1638
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

1639
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

1640
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

1641
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
1642
Q

Protease that cleave and activate caspases

A

Granzyme

1643
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

1644
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
1645
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

1646
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

1647
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

1648
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

1649
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

1650
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

1651
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

1652
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

1653
Q

Cells involved in acute humoral rejection

A

B cells and Abs

1654
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

1655
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

1656
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

1657
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

1658
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

1659
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

1660
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

1661
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

1662
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

1663
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

1664
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

1665
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
1666
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

1667
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

1668
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

1669
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

1670
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

1671
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

1672
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

1673
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
1674
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
1675
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

1676
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
1677
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

1678
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
1679
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

1680
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

1681
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

1682
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

1683
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
1684
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

1685
Q

Most common cause of death in SLE

A

Renal failure

1686
Q

Second most common cause of death in SLE

A

Sepsis/infection

1687
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
1688
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
1689
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
1690
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

1691
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
1692
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

1693
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

1694
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

1695
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

1696
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

1697
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
1698
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

1699
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

1700
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

1701
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
1702
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

1703
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

1704
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

1705
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

1706
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

1707
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

1708
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

1709
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
1710
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

1711
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

1712
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

1713
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

1714
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

1715
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

1716
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
1717
Q

Calcium deposit in the skin

A

Calcinosis

1718
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

1719
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

1720
Q

Skin thickening of the fingers

A

Sclerodactyly

1721
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

1722
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

1723
Q

Two types of immunodeficiency

A

Primary

Secondary

1724
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

1725
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

1726
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

1727
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

1728
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
1729
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

1730
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

1731
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

1732
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

1733
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

1734
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

1735
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

1736
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

1737
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

1738
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

1739
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

1740
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

1741
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

1742
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

1743
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

1744
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

1745
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

1746
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

1747
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

1748
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

1749
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

1750
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

1751
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

1752
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

1753
Q

Promote survival and differentiation of B cells

A

BAFF

1754
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

1755
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

1756
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

1757
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

1758
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

1759
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

1760
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

1761
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

1762
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

1763
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

1764
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

1765
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

1766
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
1767
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
1768
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
1769
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

1770
Q

Two major target of HIV

A

Immune system and CNS

1771
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

1772
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

1773
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

1774
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

1775
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

1776
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

1777
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

1778
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

1779
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

1780
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

1781
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

1782
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

1783
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

1784
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

1785
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

1786
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

1787
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

1788
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

1789
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

1790
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

1791
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
1792
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

1793
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

1794
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

1795
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

1796
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
1797
Q

Protease that cleave and activate caspases

A

Granzyme

1798
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

1799
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
1800
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

1801
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

1802
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

1803
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

1804
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

1805
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

1806
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

1807
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

1808
Q

Cells involved in acute humoral rejection

A

B cells and Abs

1809
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

1810
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

1811
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

1812
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

1813
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

1814
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

1815
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

1816
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

1817
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

1818
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

1819
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

1820
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
1821
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

1822
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

1823
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

1824
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

1825
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

1826
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

1827
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

1828
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
1829
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
1830
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

1831
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
1832
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

1833
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
1834
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

1835
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

1836
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

1837
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

1838
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
1839
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

1840
Q

Most common cause of death in SLE

A

Renal failure

1841
Q

Second most common cause of death in SLE

A

Sepsis/infection

1842
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
1843
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
1844
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
1845
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

1846
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
1847
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

1848
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

1849
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

1850
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

1851
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

1852
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
1853
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

1854
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

1855
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

1856
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
1857
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

1858
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

1859
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

1860
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

1861
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

1862
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

1863
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

1864
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
1865
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

1866
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

1867
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

1868
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

1869
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

1870
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

1871
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
1872
Q

Calcium deposit in the skin

A

Calcinosis

1873
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

1874
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

1875
Q

Skin thickening of the fingers

A

Sclerodactyly

1876
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

1877
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

1878
Q

Two types of immunodeficiency

A

Primary

Secondary

1879
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

1880
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

1881
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

1882
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

1883
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
1884
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

1885
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

1886
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

1887
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

1888
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

1889
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

1890
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

1891
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

1892
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

1893
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

1894
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

1895
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

1896
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

1897
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

1898
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

1899
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

1900
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

1901
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

1902
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

1903
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

1904
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

1905
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

1906
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

1907
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

1908
Q

Promote survival and differentiation of B cells

A

BAFF

1909
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

1910
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

1911
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

1912
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

1913
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

1914
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

1915
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

1916
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

1917
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

1918
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

1919
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

1920
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

1921
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
1922
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
1923
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
1924
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

1925
Q

Two major target of HIV

A

Immune system and CNS

1926
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

1927
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

1928
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

1929
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

1930
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

1931
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

1932
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

1933
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

1934
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

1935
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

1936
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

1937
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

1938
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

1939
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

1940
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

1941
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

1942
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

1943
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

1944
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

1945
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

1946
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
1947
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

1948
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

1949
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
1950
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

1951
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

1952
Q

Protease that cleave and activate caspases

A

Granzyme

1953
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

1954
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
1955
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

1956
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

1957
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

1958
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

1959
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

1960
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

1961
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

1962
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

1963
Q

Cells involved in acute humoral rejection

A

B cells and Abs

1964
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

1965
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

1966
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

1967
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

1968
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

1969
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

1970
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

1971
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

1972
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

1973
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

1974
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

1975
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
1976
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

1977
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

1978
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

1979
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

1980
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

1981
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

1982
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

1983
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
1984
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
1985
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

1986
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
1987
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

1988
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
1989
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

1990
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

1991
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

1992
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

1993
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
1994
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

1995
Q

Most common cause of death in SLE

A

Renal failure

1996
Q

Second most common cause of death in SLE

A

Sepsis/infection

1997
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
1998
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
1999
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
2000
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

2001
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
2002
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

2003
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

2004
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

2005
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

2006
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

2007
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
2008
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

2009
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

2010
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

2011
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
2012
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

2013
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

2014
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

2015
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

2016
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

2017
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

2018
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

2019
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
2020
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

2021
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

2022
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

2023
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

2024
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

2025
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

2026
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
2027
Q

Calcium deposit in the skin

A

Calcinosis

2028
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

2029
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

2030
Q

Skin thickening of the fingers

A

Sclerodactyly

2031
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

2032
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

2033
Q

Two types of immunodeficiency

A

Primary

Secondary

2034
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

2035
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

2036
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

2037
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

2038
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
2039
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

2040
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

2041
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

2042
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

2043
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

2044
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

2045
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

2046
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

2047
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

2048
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

2049
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

2050
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

2051
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

2052
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

2053
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

2054
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

2055
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

2056
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

2057
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

2058
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

2059
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

2060
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

2061
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

2062
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

2063
Q

Promote survival and differentiation of B cells

A

BAFF

2064
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

2065
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

2066
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

2067
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

2068
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

2069
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

2070
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

2071
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

2072
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

2073
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

2074
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

2075
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

2076
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
2077
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
2078
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
2079
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

2080
Q

Two major target of HIV

A

Immune system and CNS

2081
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

2082
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

2083
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

2084
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

2085
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

2086
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

2087
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

2088
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

2089
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

2090
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

2091
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

2092
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

2093
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

2094
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

2095
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

2096
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

2097
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

2098
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

2099
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

2100
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

2101
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
2102
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

2103
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

2104
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

2105
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

2106
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
2107
Q

Protease that cleave and activate caspases

A

Granzyme

2108
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

2109
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
2110
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

2111
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

2112
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

2113
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

2114
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

2115
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

2116
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

2117
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

2118
Q

Cells involved in acute humoral rejection

A

B cells and Abs

2119
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

2120
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

2121
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

2122
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

2123
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

2124
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

2125
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

2126
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

2127
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

2128
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

2129
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

2130
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
2131
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

2132
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

2133
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

2134
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

2135
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

2136
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

2137
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

2138
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
2139
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
2140
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

2141
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
2142
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

2143
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
2144
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

2145
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

2146
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

2147
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

2148
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
2149
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

2150
Q

Most common cause of death in SLE

A

Renal failure

2151
Q

Second most common cause of death in SLE

A

Sepsis/infection

2152
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
2153
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
2154
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
2155
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

2156
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
2157
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

2158
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

2159
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

2160
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

2161
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

2162
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
2163
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

2164
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

2165
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

2166
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
2167
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

2168
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

2169
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

2170
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

2171
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

2172
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

2173
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

2174
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
2175
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

2176
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

2177
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

2178
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

2179
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

2180
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

2181
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
2182
Q

Calcium deposit in the skin

A

Calcinosis

2183
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

2184
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

2185
Q

Skin thickening of the fingers

A

Sclerodactyly

2186
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

2187
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

2188
Q

Two types of immunodeficiency

A

Primary

Secondary

2189
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

2190
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

2191
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

2192
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

2193
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
2194
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

2195
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

2196
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

2197
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

2198
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

2199
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

2200
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

2201
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

2202
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

2203
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

2204
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

2205
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

2206
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

2207
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

2208
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

2209
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

2210
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

2211
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

2212
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

2213
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

2214
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

2215
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

2216
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

2217
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

2218
Q

Promote survival and differentiation of B cells

A

BAFF

2219
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

2220
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

2221
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

2222
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

2223
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

2224
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

2225
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

2226
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

2227
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

2228
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

2229
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

2230
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

2231
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
2232
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
2233
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
2234
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

2235
Q

Two major target of HIV

A

Immune system and CNS

2236
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

2237
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

2238
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

2239
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

2240
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

2241
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

2242
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

2243
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

2244
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

2245
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

2246
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

2247
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

2248
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

2249
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

2250
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

2251
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

2252
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

2253
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

2254
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

2255
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

2256
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
2257
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

2258
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

2259
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

2260
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

2261
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
2262
Q

Protease that cleave and activate caspases

A

Granzyme

2263
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

2264
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
2265
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

2266
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

2267
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

2268
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

2269
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

2270
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

2271
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

2272
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

2273
Q

Cells involved in acute humoral rejection

A

B cells and Abs

2274
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

2275
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

2276
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

2277
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

2278
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

2279
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

2280
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

2281
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

2282
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

2283
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

2284
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

2285
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
2286
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

2287
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

2288
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

2289
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

2290
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

2291
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

2292
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

2293
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
2294
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
2295
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

2296
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
2297
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

2298
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
2299
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

2300
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

2301
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

2302
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

2303
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
2304
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

2305
Q

Most common cause of death in SLE

A

Renal failure

2306
Q

Second most common cause of death in SLE

A

Sepsis/infection

2307
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
2308
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
2309
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
2310
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

2311
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
2312
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

2313
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

2314
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

2315
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

2316
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

2317
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
2318
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

2319
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

2320
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

2321
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
2322
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

2323
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

2324
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

2325
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

2326
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

2327
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

2328
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

2329
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
2330
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

2331
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

2332
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

2333
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

2334
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

2335
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

2336
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
2337
Q

Calcium deposit in the skin

A

Calcinosis

2338
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

2339
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

2340
Q

Skin thickening of the fingers

A

Sclerodactyly

2341
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

2342
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

2343
Q

Two types of immunodeficiency

A

Primary

Secondary

2344
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

2345
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

2346
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

2347
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

2348
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
2349
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

2350
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

2351
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

2352
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

2353
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

2354
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

2355
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

2356
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

2357
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

2358
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

2359
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

2360
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

2361
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

2362
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

2363
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

2364
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

2365
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

2366
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

2367
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

2368
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

2369
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

2370
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

2371
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

2372
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

2373
Q

Promote survival and differentiation of B cells

A

BAFF

2374
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

2375
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

2376
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

2377
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

2378
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

2379
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

2380
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

2381
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

2382
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

2383
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

2384
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

2385
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

2386
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
2387
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
2388
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
2389
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

2390
Q

Two major target of HIV

A

Immune system and CNS

2391
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

2392
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

2393
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

2394
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

2395
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

2396
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

2397
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

2398
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

2399
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

2400
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

2401
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

2402
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

2403
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

2404
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

2405
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

2406
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

2407
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

2408
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

2409
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

2410
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

2411
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
2412
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

2413
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

2414
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

2415
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

2416
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
2417
Q

Protease that cleave and activate caspases

A

Granzyme

2418
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

2419
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
2420
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

2421
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

2422
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

2423
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

2424
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

2425
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

2426
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

2427
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

2428
Q

Cells involved in acute humoral rejection

A

B cells and Abs

2429
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

2430
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

2431
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

2432
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

2433
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

2434
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

2435
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

2436
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

2437
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

2438
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

2439
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

2440
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
2441
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

2442
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

2443
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

2444
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

2445
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

2446
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

2447
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

2448
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
2449
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
2450
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

2451
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
2452
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

2453
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
2454
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

2455
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

2456
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

2457
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

2458
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
2459
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

2460
Q

Most common cause of death in SLE

A

Renal failure

2461
Q

Second most common cause of death in SLE

A

Sepsis/infection

2462
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
2463
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
2464
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
2465
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

2466
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
2467
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

2468
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

2469
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

2470
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

2471
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

2472
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
2473
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

2474
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

2475
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

2476
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
2477
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

2478
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

2479
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

2480
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

2481
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

2482
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

2483
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

2484
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
2485
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

2486
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

2487
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

2488
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

2489
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

2490
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

2491
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
2492
Q

Calcium deposit in the skin

A

Calcinosis

2493
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

2494
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

2495
Q

Skin thickening of the fingers

A

Sclerodactyly

2496
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

2497
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

2498
Q

Two types of immunodeficiency

A

Primary

Secondary

2499
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

2500
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

2501
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

2502
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

2503
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
2504
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

2505
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

2506
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

2507
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

2508
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

2509
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

2510
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

2511
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

2512
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

2513
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

2514
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

2515
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

2516
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

2517
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

2518
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

2519
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

2520
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

2521
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

2522
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

2523
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

2524
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

2525
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

2526
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

2527
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

2528
Q

Promote survival and differentiation of B cells

A

BAFF

2529
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

2530
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

2531
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

2532
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

2533
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

2534
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

2535
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

2536
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

2537
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

2538
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

2539
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

2540
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

2541
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
2542
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
2543
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
2544
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

2545
Q

Two major target of HIV

A

Immune system and CNS

2546
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

2547
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

2548
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

2549
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

2550
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

2551
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

2552
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

2553
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

2554
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

2555
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

2556
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

2557
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

2558
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

2559
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

2560
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

2561
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

2562
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

2563
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

2564
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

2565
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

2566
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
2567
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

2568
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

2569
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

2570
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

2571
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
2572
Q

Protease that cleave and activate caspases

A

Granzyme

2573
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

2574
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
2575
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

2576
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

2577
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

2578
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

2579
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

2580
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

2581
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

2582
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

2583
Q

Cells involved in acute humoral rejection

A

B cells and Abs

2584
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

2585
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

2586
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

2587
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

2588
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

2589
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

2590
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

2591
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

2592
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

2593
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

2594
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

2595
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
2596
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

2597
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

2598
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

2599
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

2600
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

2601
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

2602
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

2603
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
2604
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
2605
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

2606
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
2607
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

2608
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
2609
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

2610
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

2611
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

2612
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

2613
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
2614
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

2615
Q

Most common cause of death in SLE

A

Renal failure

2616
Q

Second most common cause of death in SLE

A

Sepsis/infection

2617
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
2618
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
2619
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
2620
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

2621
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
2622
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

2623
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

2624
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

2625
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

2626
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

2627
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
2628
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

2629
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

2630
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

2631
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
2632
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

2633
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

2634
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

2635
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

2636
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

2637
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

2638
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

2639
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
2640
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

2641
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

2642
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

2643
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

2644
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

2645
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

2646
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
2647
Q

Calcium deposit in the skin

A

Calcinosis

2648
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

2649
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

2650
Q

Skin thickening of the fingers

A

Sclerodactyly

2651
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

2652
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

2653
Q

Two types of immunodeficiency

A

Primary

Secondary

2654
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

2655
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

2656
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

2657
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

2658
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
2659
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

2660
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

2661
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

2662
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

2663
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

2664
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

2665
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

2666
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

2667
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

2668
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

2669
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

2670
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

2671
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

2672
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

2673
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

2674
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

2675
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

2676
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

2677
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

2678
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

2679
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

2680
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

2681
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

2682
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

2683
Q

Promote survival and differentiation of B cells

A

BAFF

2684
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

2685
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

2686
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

2687
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

2688
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

2689
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

2690
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

2691
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

2692
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

2693
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

2694
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

2695
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

2696
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
2697
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
2698
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
2699
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

2700
Q

Two major target of HIV

A

Immune system and CNS

2701
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

2702
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

2703
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

2704
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

2705
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

2706
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

2707
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

2708
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

2709
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

2710
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

2711
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

2712
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

2713
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

2714
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

2715
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

2716
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

2717
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

2718
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

2719
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

2720
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

2721
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
2722
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

2723
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

2724
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

2725
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

2726
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
2727
Q

Protease that cleave and activate caspases

A

Granzyme

2728
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

2729
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
2730
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

2731
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

2732
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

2733
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

2734
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

2735
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

2736
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

2737
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

2738
Q

Cells involved in acute humoral rejection

A

B cells and Abs

2739
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

2740
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

2741
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

2742
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

2743
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

2744
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

2745
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

2746
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

2747
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

2748
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

2749
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

2750
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
2751
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

2752
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

2753
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

2754
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

2755
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

2756
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

2757
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

2758
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
2759
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
2760
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

2761
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
2762
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

2763
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
2764
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

2765
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

2766
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

2767
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

2768
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
2769
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

2770
Q

Most common cause of death in SLE

A

Renal failure

2771
Q

Second most common cause of death in SLE

A

Sepsis/infection

2772
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
2773
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
2774
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
2775
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

2776
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
2777
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

2778
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

2779
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

2780
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

2781
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

2782
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
2783
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

2784
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

2785
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

2786
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
2787
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

2788
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

2789
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

2790
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

2791
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

2792
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

2793
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

2794
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
2795
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

2796
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

2797
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

2798
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

2799
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

2800
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

2801
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
2802
Q

Calcium deposit in the skin

A

Calcinosis

2803
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

2804
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

2805
Q

Skin thickening of the fingers

A

Sclerodactyly

2806
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

2807
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

2808
Q

Two types of immunodeficiency

A

Primary

Secondary

2809
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

2810
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

2811
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

2812
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

2813
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
2814
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

2815
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

2816
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

2817
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

2818
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

2819
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

2820
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

2821
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

2822
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

2823
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

2824
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

2825
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

2826
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

2827
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

2828
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

2829
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

2830
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

2831
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

2832
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

2833
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

2834
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

2835
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

2836
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

2837
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

2838
Q

Promote survival and differentiation of B cells

A

BAFF

2839
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

2840
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

2841
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

2842
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

2843
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

2844
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

2845
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

2846
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

2847
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

2848
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

2849
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

2850
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

2851
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
2852
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
2853
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
2854
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

2855
Q

Two major target of HIV

A

Immune system and CNS

2856
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

2857
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

2858
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

2859
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

2860
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

2861
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

2862
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

2863
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

2864
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

2865
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

2866
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

2867
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

2868
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

2869
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

2870
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

2871
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

2872
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

2873
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

2874
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

2875
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

2876
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
2877
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

2878
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

2879
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

2880
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

2881
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
2882
Q

Protease that cleave and activate caspases

A

Granzyme

2883
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

2884
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
2885
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

2886
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

2887
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

2888
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

2889
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

2890
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

2891
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

2892
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

2893
Q

Cells involved in acute humoral rejection

A

B cells and Abs

2894
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

2895
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

2896
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

2897
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

2898
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

2899
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

2900
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

2901
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

2902
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

2903
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

2904
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

2905
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
2906
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

2907
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

2908
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

2909
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

2910
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

2911
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

2912
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

2913
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
2914
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
2915
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

2916
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
2917
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

2918
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
2919
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

2920
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

2921
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

2922
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

2923
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
2924
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

2925
Q

Most common cause of death in SLE

A

Renal failure

2926
Q

Second most common cause of death in SLE

A

Sepsis/infection

2927
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
2928
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
2929
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
2930
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

2931
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
2932
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

2933
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

2934
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

2935
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

2936
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

2937
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
2938
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

2939
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

2940
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

2941
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
2942
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

2943
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

2944
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

2945
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

2946
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

2947
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

2948
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

2949
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
2950
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

2951
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

2952
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

2953
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

2954
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

2955
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

2956
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
2957
Q

Calcium deposit in the skin

A

Calcinosis

2958
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

2959
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

2960
Q

Skin thickening of the fingers

A

Sclerodactyly

2961
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

2962
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

2963
Q

Two types of immunodeficiency

A

Primary

Secondary

2964
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

2965
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

2966
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

2967
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

2968
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
2969
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

2970
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

2971
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

2972
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

2973
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

2974
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

2975
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

2976
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

2977
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

2978
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

2979
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

2980
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

2981
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

2982
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

2983
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

2984
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

2985
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

2986
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

2987
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

2988
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

2989
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

2990
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

2991
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

2992
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

2993
Q

Promote survival and differentiation of B cells

A

BAFF

2994
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

2995
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

2996
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

2997
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

2998
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

2999
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

3000
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

3001
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

3002
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

3003
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

3004
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

3005
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

3006
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
3007
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
3008
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
3009
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

3010
Q

Two major target of HIV

A

Immune system and CNS

3011
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

3012
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

3013
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

3014
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

3015
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

3016
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

3017
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

3018
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

3019
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

3020
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

3021
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

3022
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

3023
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

3024
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

3025
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

3026
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

3027
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

3028
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

3029
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

3030
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

3031
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
3032
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

3033
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

3034
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

3035
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

3036
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
3037
Q

Protease that cleave and activate caspases

A

Granzyme

3038
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

3039
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
3040
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

3041
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

3042
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

3043
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

3044
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

3045
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

3046
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

3047
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

3048
Q

Cells involved in acute humoral rejection

A

B cells and Abs

3049
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

3050
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

3051
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

3052
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

3053
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

3054
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

3055
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

3056
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

3057
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

3058
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

3059
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

3060
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
3061
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

3062
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

3063
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

3064
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

3065
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

3066
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

3067
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

3068
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
3069
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
3070
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

3071
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
3072
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

3073
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
3074
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

3075
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

3076
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

3077
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

3078
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
3079
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

3080
Q

Most common cause of death in SLE

A

Renal failure

3081
Q

Second most common cause of death in SLE

A

Sepsis/infection

3082
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
3083
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
3084
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
3085
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

3086
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
3087
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

3088
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

3089
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

3090
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

3091
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

3092
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
3093
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

3094
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

3095
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

3096
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
3097
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

3098
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

3099
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

3100
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

3101
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

3102
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

3103
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

3104
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
3105
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

3106
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

3107
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

3108
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

3109
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

3110
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

3111
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
3112
Q

Calcium deposit in the skin

A

Calcinosis

3113
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

3114
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

3115
Q

Skin thickening of the fingers

A

Sclerodactyly

3116
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

3117
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

3118
Q

Two types of immunodeficiency

A

Primary

Secondary

3119
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

3120
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

3121
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

3122
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

3123
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
3124
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

3125
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

3126
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

3127
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

3128
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

3129
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

3130
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

3131
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

3132
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

3133
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

3134
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

3135
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

3136
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

3137
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

3138
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

3139
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

3140
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

3141
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

3142
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

3143
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

3144
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

3145
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

3146
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

3147
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

3148
Q

Promote survival and differentiation of B cells

A

BAFF

3149
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

3150
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

3151
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

3152
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

3153
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

3154
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

3155
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

3156
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

3157
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

3158
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

3159
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

3160
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

3161
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
3162
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
3163
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
3164
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

3165
Q

Two major target of HIV

A

Immune system and CNS

3166
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

3167
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

3168
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

3169
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

3170
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

3171
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

3172
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

3173
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

3174
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

3175
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

3176
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

3177
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

3178
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

3179
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

3180
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

3181
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

3182
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

3183
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

3184
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

3185
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

3186
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
3187
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

3188
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

3189
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

3190
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

3191
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis
3192
Q

Protease that cleave and activate caspases

A

Granzyme

3193
Q

End result of T-cell mediated cytotoxicity

A

Apoptosis

3194
Q

Three types of transplant rejection of kidney cells

A
  1. Hyperacute rejection
  2. Acute rejection
  3. Chronic rejection
3195
Q

Type of rejection that occurs minute or hours after transplantation due to pre-formed anti-donor Abs present in the circulation of the recipient

A

Hyperacute rejection

3196
Q

Gross appearance of hyperacute rejection

A

Mottled, cyanotic, flaccid kidney

Pale, hyperemic areas with white infarct

3197
Q

Two subtypes of acute rejection

A

Acute cellular rejection

Acute humoral rejection

3198
Q

Type of rejection that occurs a few days after cessation of immunosuppressive therapy

A

Acute rejection

3199
Q

Subtype of acute rejection that characterized by interstitial mononuclear infiltrate

A

Acute cellular rejection

3200
Q

Subtype of acute rejection that is characterized by necrotizing vasculitis with endothelial cell necrosis causing extensive necrosis of renal parenchyma

A

Acute humoral rejection

3201
Q

Cells involved in acute cellular rejection

A

CD4+ and cytotoxic T cells: damage tubular and vascular endothelial cells
CD8+ T cells: recruits cytokines causing inflammation that damages the graft, finally resulting to vascular cleavage

3202
Q

Manifestations of acute rejection

A

Damage to glomeruli and blood vessels
Inflammation of glomeruli and peritubular capillaries
Deposition of complement products

3203
Q

Cells involved in acute humoral rejection

A

B cells and Abs

3204
Q

Type of rejection that occurs after months to years after transplantation

A

Chronic rejection

3205
Q

Morphology of chronic rejection

A

Vascular changes: Obliterative intimal fibrosis
Interstitial fibrosis
Tubular atrophy with loss of renal parenchyma

3206
Q

Clinical presentation of chronic rejection

A

Progressive organ dysfunction

3207
Q

Cytokines that differentiate CD4+ T cells into TH1

A

IL-12, IFN-gamma

3208
Q

Cytokines that differentiate CD4+ T cells into TH17

A

IL-1, IL-16 and IL-23

3209
Q

TH cell subset that recruits more macrophages mononuclear cells

A

TH1

3210
Q

TH cell subset that recruits neutrophils and monocytes creating a more neutrophilic appearance

A

TH17

3211
Q

Prototype disorders of Type I: Immediate hypersensitivity reactions

A

Anaphylaxis; allergies, bronchial asthma (atopic forms)

3212
Q

Prototype disorders of Type II: Ab-mediated hypersensitivity

A

AIHA (IIa)
Goodpasture syndrome (IIb)
Graves, Myasthenia Gravis (IIc)

3213
Q

Prototype disorders of Type III: Immune-complex mediated hypersensitivity

A

SLE
Some forms of Glomerulonephritis
Serum sickness
Arthus reaction

3214
Q

Prototype disorders of Type IV: Cell-mediated hypersensitivity

A

Tuberculosis (IVa)
Response to viral infections (IVb)
Transplant rejection

3215
Q

Disease examples of cell-mediated hypersensitivity reactions

A 
Rheumatoid arthritis
Multiple sclerosis
DM type I
Inflammatory bowel disease
Psoriasis
Contact sensitivity
3216
Q

Rheumatoid arthritis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Collagen and citrullinated self proteins

Manifestation: Chronic arthritis with inflammation, destruction of articular cartilage

3217
Q

Multiple sclerosis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Protein Ag in myelin

Manifestation: Demyelination in CNS with perivascular inflammation; paralysis

3218
Q

DM type I
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Ag of pancreatic islets of B cells (insulin, glutamic acid decaraboxylase, etc)
Manifestation: Insulitis (chronic inflammation in islets), destruction of active cells; diabetes

3219
Q

Inflammatory bowel disease
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Enteric disease; bacteria; self Ag

Manifestation: Chronic intestinal inflammation or obstruction

3220
Q

Psoriasis
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Unknown

Manifestation: Destructive plaques on the skin

3221
Q

Contact sensitivity
Target Ag: ____________
Manifestation: _____________

A

Target Ag: Various environment chemicals (e.g. urushiol from poison ivy or oak); Therapeutic drugs
Manifestation: Epidermal necrosis, dermal inflammation skin rash and blisters

3222
Q

These result from tissue injury cause by T cells or Abs that react against self-antigens

A

Autoimmune diseases

3223
Q

Features of autoimmune diseases in general

A
  1. Female predilection
  2. Characterized by remissions and exacerbations
  3. Increased incidence of malignancy
  4. Familial prevalence of the same or other A.I.D.
  5. Clinical and serologic overlaps
  6. Patients often have increase immunoglobulin in the serum
3224
Q

Autoimmune disease may arise from combination of

A
  1. Inheritance of susceptibility genes which contribute to breakdown of self-tolerance
  2. Environmental triggers like infections and tissue damage which mimics endogenous proteins
  3. Promotion of the activation of self-reactive lymphocytes
3225
Q

Organ specific spectrum of autoimmune diseases

A

Ab directed against a single organ/tissue

Localized lesions

3226
Q

Examples of organ specific spectrum of autoimmune diseases

A 
Hasimoto’s thyroiditis
Pernicios anemia
Thyrotoxicosis (Graves’ disease)
Autoimmune hemolytic anemia (AIHA)
Immune thromocytopenic purpura (ITP)
Insulin-dependent diabetes mellitus (IDDM)
3227
Q

Non-organ specific spectrum of autoimmune diseases

A

Ab not directed to a single organ/tissue

Widespread lesions

3228
Q

Example of organ specific spectrum of autoimmune diseases

A 
SLE
Sjorgren syndrome
Scleroderma
Rheumatoid arthritis
Inflammatory myopathies
Mixed connective tissue disease
3229
Q

Rare autoimmune in which which the antibodies attack the basement membrane of the glomerulus and alveoli causing pulmonary haemorrhage and kidney failure

A

Goodpasture syndrome

3230
Q

Chronic, repeating relapsing illness characterized by injury to the skin, joints, kidney and basement membrane (areas with high blood flow)

A

SLE

3231
Q

Affects multiple organs due to a wast array of autoAbs, particularly anti-nucleus Abs

A

SLE

3232
Q

Clinical feature of SLE

A
  1. More common in females (10:1 - 20:1)

2. 2nd-3rd decade: acute, more omninous; Older: more insidious, better prognosis

3233
Q

Most common signs and symptoms of SLE

A
  1. Hematologic - 100%
  2. Musculo-skeletal (arthritis) - 90%
  3. Skin (Butterfly rash) - 85%
  4. Fever - 83% (55-85%)
  5. Renal, pulmonary, cardiac - 30-50%
3234
Q

Course of SLE

A

Acute: death within weeks to months
Chronic: with treatment, 10-20 years

3235
Q

Most common cause of death in SLE

A

Renal failure

3236
Q

Second most common cause of death in SLE

A

Sepsis/infection

3237
Q

Some factors related to pathogenesis of SLE

A
  1. Genetic: IgA, C2 deficiency; greater chance in family groups associated with certain halotypes (most common)
  2. Environmental: drugs, UV light, hormones (stimulate formation of Abs against DNA)
  3. Immunologic: defective elimination of self-reactive B cells in the bone marrow, CD4+ T cells specific for nucleosomal Ag escape tolerance
3238
Q

Classification scheme in diagnosing SLE

A
  1. Patients has four or more clinical and immunologic criteria present (with at least one clinical and one immunologic)
  2. Demonstrate presence of Ab to Anti-DNA (more specific)
3239
Q

Three mechanisms of tissue damage in SLE

A
  1. Immune complex disease (Type III)
  2. Ab directed against cell type (Type II)
  3. Presence of Antiphospholipid Antibodies (Secondary to APAS)
3240
Q

Mechanism of immune complex disease in SLE

A

Ab against DNA
Ab to histones
Ab to nonhistone proteins bound to RNA
Ab to nuclear Ag

3241
Q

Clinical manifestation of immune complex disease in SLE

A
  1. Vasculitis
  2. Glomerulonephritis
  3. Arthritis
  4. Heart
  5. Skin
  6. Others: Interstitial pneumonitis, cerebral infarcts and hemorrhages, pericariditis
3242
Q

Non-erosive synovitis with little joint deformity in SLE

A

Arthritis

3243
Q

Affects small arteries and arterioles (in spleen: onion-skin lesions) in SLE

A

Vasculitis

3244
Q

Endocarditis in SLE characterized by 1-3mm warty deposits on any valve, also called vegetative

A

Liebmann-Sacks endocarditis

3245
Q

A sign of SLE seen histologically as
H&E: liquefactive degeneration of basal layer of epidermis and edema at the D-E junction
IF: Ig and complement deposits in D-E junction

A

Malar rash

3246
Q

Mechanism of Ab mediated disease

A

Ab against RBCs (anemia)
Ab against WBC (leukopenia)
Ab against platelets (thrombocytopenia)

3247
Q

Patterns of FANA

A
  1. Homogenous (anti-DNA protein)
  2. Peripheral (anti-nucleolar DNA)
  3. Nucleolar (anti-nucleolar RNA)
  4. Speckled (anti-ENA)
3248
Q

Characteristic FANA pattern of SLE

A

Peripheral pattern

3249
Q 
Present in 40-50% of SLE patients
Bind to cardiolipin Ag which is used in syphilis testing (false positive)
Predisposed thrombosis (venous and arterial; deep vein thrombosis)
A

Anti-phospholipid Ab

3250
Q

Neutrophil or macrophage that has phagocytosed the denature nuclear material or Ab-coated nucleus of another cell

A

LE cell

3251
Q

Typical features of SLE

A
  1. History and PE: young female with malar rash, fever, joint pains, hematologic problem
  2. (+) ANA: peripheral pattern
  3. Ab to dsDNA and Smith Ag
  4. (+) Lupus band test on skin biopsy
  5. Decrease complement level: C3
  6. Renal biopsy shows glomerulonephritis and immune complex deposits by immunoflourescence
3252
Q

Chronic inflammatory disease characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands

A

Sjogren Syndrome

3253
Q

Primary form or isolated disorder of Sjorgen syndrome

A

Sicca Syndrome

3254
Q

Most common autoimmune disease associated with another autoimmune diseases

A

Rheumatoid arthritis (75% have rheumatoid factor)

3255
Q

90% have Abs directed to ribonucleoprotein antigens SS-A (Ro) and SS-B (LA)

A

Secondary form

3256
Q

Sjogren Syndrome in association with another autoimmune disease

A

Secondary form

3257
Q

Dry eyes that causes blurring of vision, burning and itching, thick secretions in the conjunctival sac

A

Keratoconjunctivitis

3258
Q

Difficulty in swallowing, decreased ability to taste, cracks and fissures in the mouth, dryness of buccal mucosa

A

Xerostomia

3259
Q

Clinical feature of Sjogren syndrome

A
  1. Most common in 50-60 year old women
  2. Keratoconjunctivitis
  3. Xerostomia
  4. Others: parotid gland enlargement (50%), dryness of the nasal mucosa, epistaxis, recurrent bronchitis and pneumonitis
  5. Increased risk of developing lymphoid malignancies
  6. Extraglandular disease in 1/3 of patients manifested as synovitis, diffuse pulmonary fibrosis and peripheral neuropathy
3260
Q

Most common type lymphoid malignancy in Sjogren syndrome

A

Marginal Zone lymphoma

3261
Q

Abnormal accumulation of fibrous tissue in the skin and multiple organs

A

Systemic sclerosis (Scleroderma)

3262
Q

Characterized by progressive fibrosis in multiple tissues, obliterate vascular disease and evidence of autoimmunity, mainly the production of multiple autoantibodies

A

Scleroderma

3263
Q

Two major categories of Scleroderma

A

Diffuse scleroderma

Limited scleroderma

3264
Q

Widespread involvement at onset with rapid progression and early visceral involvement (GIT and lungs)

A

Diffuse scleroderma

3265
Q

Skin involvement confined to fingers, forearm and face with late visceral involvement

A

Limited scleroderma

3266
Q

Symptoms of limited type scleroderma

A 
CREST syndrome
C-alcinosis
R-aynaud phenomenon
E-sophageal dysmotility
S-clerodactyly
T-elangiectasia
3267
Q

Calcium deposit in the skin

A

Calcinosis

3268
Q

Exaggerated type of vasocontriction in the hands with the fingers undergoing characteristic pallor and hypereremia when exposed to cold

A

Raynaud phenomenon

3269
Q

Difficulty in swallowing because of fibrosis and sclerosis of the esophagus due to chronic inflammation

A

Esophageal dymotility

3270
Q

Skin thickening of the fingers

A

Sclerodactyly

3271
Q

Dilatation of capillaries of the hands, face and mucous membrane presenting a spider-like appearance (spider veins)

A

Telangiectasia

3272
Q

Suppressed immune system which may be caused by inherited defects affecting the immune system development or secondary to other diseases

A

Immunodeficiency

3273
Q

Two types of immunodeficiency

A

Primary

Secondary

3274
Q

Type of immunodeficiency which is almost always genetically determined, usually X-linked, seen in infancy (6 months-2 years) and associated with recurrent infections

A

Primary immunodeficiency

3275
Q

Acquired type of immunodeficiency which result from altered immune function caused by malnutrition, viral infection, irradiation, use of immunosuppressive drugs, lymphoproliferative diseases

A

Secondary immunodeficiency

3276
Q

Most common causes of secondary immunodeficiency

A

Chemotherapy and radiotherapy

3277
Q

Differentiation of mature plasma cells which requires T cells

A

Class switching

3278
Q

Primary immunodeficiency is based on deficient components of the immune system

A
  1. Stem cell deficiency
  2. B-cells deficiency
  3. T-cell deficiency
  4. Deficiency of myeloid elements
  5. Complement deficiency
3279
Q

Example of primary type diseases

A

B-cell deficiency: Bruton’s X-linked Agammaglobulinemia
T-cell deficiency: DiGeorge syndrome
Stem cell defect: Sever Combines Immunodeficiency (SCID)
Complement deficiency
Others: CVID, Hyper IgM syndrome, Wiscott-Alrich Syndrome, X-linked Lymphoproliferative disorder, Isolated IgA deficiency

3280
Q 
B-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: Normal
Susceptibility to infections: Pyogenic bacteria (Staphylococcus, Pneumococcus, etc)
Treatment: Gamma-globulin

3281
Q

Abnormality in projection of the BTK gene (Bruton tyrosine kinase) which is responsible for sending maturation signals from the pre-B-cells and B cell receptors

A

Bruton’s X-linked agammaglobulinemia

3282
Q

Failure of B cell maturation and absence of gammaglobulins

A

Bruton’s X-linked agammaglobulinemia

3283
Q

Pathologic finding of B-cell deficiency

A

B-cells almost absent in lymphocytes, spleen, bone marrow and connective tissues
Germinal centers in the lymph nodes, Peyer’s patches, appendix and tonsils are underdeveloped

3284
Q

Features of Bruton’s X-linked agammaglobulinemia

A

Lack of mature B cells in the circulation
Serum levels of all Igs are depressed
T cell numbers and function are normal

3285
Q 
T-cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: Intracellular microbes (Virus, Fungi, TB)
Treatment: Thymus graft

3286
Q

Pathologic findings of T-cell deficiency

A

Low circulating T-lymphocytes
Depleted T-dependent paracortical ares of the lymph node and T-dependent areas of the spleen
Plasma cells are normal in number in lymphoid tissues

3287
Q

Failure of the development of the 3rd and 4th pharyngeal pouches

A

Di George Syndrome

3288
Q

Features of DiGeorge Syndrome

A

Thymic hypoplasia or aplasia: T cell deficiency
Parathyroid hypoplasia: Tetany
Congenital defects of the heart and great vessels (due to deletion of gene that maps Ch22q11)
Dysmorphic fascies

3289
Q 
Stem cell deficiency
Humoral response: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Cell-mediated response: \_\_\_\_\_\_\_\_\_\_\_\_
Susceptibility to infections: \_\_\_\_\_\_\_\_\_\_\_\_
Treatment: \_\_\_\_\_\_\_\_\_\_\_\_
A

Humoral response: ⬇️⬇️
Cell-mediated response: ⬇️⬇️
Susceptibility to infections: All types
Treatment: Bone marrow graft

3290
Q

Pathologic findings of stem cell deficiency

A

Absence of T and B cells in the blood, lymph nodes and spleen
Thymus devoid of lymphoid cells or Hassall’s corpuscles

3291
Q

Two types of SCID

A

X-linked SCID

Adenosine Deaminase deficiency

3292
Q

Type of SCID that is found in 50-60% of cases caused by mutations of common gamma chain of the subunit of the cytokine receptor

A

X-linked SCID

3293
Q

Clinical manifestations of X-linked SCID

A

Pro-T cells cannot differentiate into immature T-cells
Normal number of B-cells but inability to produce Ig due to inhibited class-switching
Thymus contains lobules of undifferentiated epithelial cells that resembles fetal cells

3294
Q

Autosomal recessive type of SCID

A

Adenosine Deaminase deficiency

3295
Q

This enzyme reduces the synthesis of deoxyadenosine and its derivatives which are toxic to rapidly dividing cells

A

ADA deficiency

3296
Q

Clinical manifestations of ADA deficiency

A

Susceptible to all types of infections
Absence of T and B cells in the blood, lymph nodes and spleen (humoral and cellular immunity are affected)
Mature looking but small thymus with remnants of Hassal’s corpuscles

3297
Q

Symptoms of ADA deficiency

A

Oral thrush
Extensive diaper rash at birth
Failure to thrive

3298
Q

DiGeorge syndrome vs. SCID

A

DiGeorge syndrome: Failure of immature T cells to develop into mature ones

SCID: failure of pro-T cells to develop into immature ones (block is at an earlier phase of development)

3299
Q

Bruton’s X-linked agammaglobulinemia vs. CVID

A

Bruton’s X-linked agammaglobulinemia: almost no B cell proliferation ➡️ agammaglobulinemia ➡️ B cell containing areas in the lymph nodes are hypoplastic

CVID: B cell proliferation with out differentiation into plasma cells ➡️ no feedback inhibition of B cell proliferation rendered by Igs ➡️ B cell containing areas in the lymph nodes are hyperplastic; later onset

3300
Q

Common Variable Immunodeficiency (CVID)
Pathology: ____________
Features: ______________

A

Pathology: abnormality in cytokine BAFF receptor
Features: affects both sexes, hypogammaglobulinemia, impaired Ab response to infection or vaccination, increase susceptibility to infections

3301
Q

Hyper IgM syndrome
Pathology: ____________
Features: ______________

A

Pathology: failure in class-switchig due to mutation on gene encoding for CD40L
Features: Absent IgA and IgE, very low IgG, susceptible to recurrent pyogenic infections, 70% X-linked, 30% autosomal recessive

3302
Q

Wiscott-Aldrich syndrome
Pathology: ____________
Features: ______________
Treatment: ____________

A

Pathology: X-linked recessive disease, mutations ion gene encoding for WASP on Xp11.23

Features: unable to produce Ab against polysaccharide Ag and poor response against protein Ag, susceptible to infection with encapsulated pyogenic bacteria, low serum IgM with normal IgG and IgA but increased IgE

Treatment: Bone marrow transplantation

3303
Q

Promote survival and differentiation of B cells

A

BAFF

3304
Q

Believed to link membrane receptors to cytoskeletal elements and is involved in cytoskeleton dependent responses such as migration and signal transduction

A

WASP

3305
Q

X-linked lymphoproliferative disorder
Pathology: ____________
Features: ______________

A

Pathology: Inability to eliminates Epstein-barr virus (EBV) causing infectious mononucleosis and development of B-cell tumors

Features: Inability to form germinal centers, produce high affinity abnormalities (Ab unable of attacking viruses), not susceptible to other viral infections besides EBV, 80% due to mutation in SAP leading to attenuated NK and T cell activation and susceptibility to viral infections

3306
Q

Isolated IgA deficiency
Pathology: ____________
Features: ______________

A

Pathology: Low levels of both serum and secretory IgA due to impaired differentiation of B cells

Features: Familial or acquired (measles or toxoplasmosis), sever anaphylactic secretion to transfusion of IgA containing blood because IgA is recognized as foreign, lack of IgA, asymptomptomatic but secretory defenses are weakened, susceptibility to respiratory, GIT and congenital infections

3307
Q

Most common form of primary immunoglobulin deficiency

A

Isolated IgA deficiency

3308
Q

Major Ig in mucosal secretions involved in defending the airways and GIT

A

IgA

3309
Q

Clinical feature of complement deficiency

A

Associated with increase susceptibility to bacterial infections (C3 deficiency)
High incidence of CT diseases (C2 and C4 deficiency with SLE)

3310
Q

Common complement deficiencies

A

C1 inhibitor
C2
C2
C5-9

3311
Q

Clinical manifestation of C1 inhibitor deficiency

A

Angioneurotic edema

3312
Q

Clinical manifestation of C2 deficiency

A

Associated with CT diseases in SLE

3313
Q

Clinical manifestation of C2 deficiency

A

Associated with bacterial infections

3314
Q

Clinical manifestation of C5-9 deficiency

A

Associated with repeated Neisseria infections and increased risk for meningitis and gonorrhea

3315
Q

Prototype of secondary type diseases

A

Acquired Immunodeficiency Syndrome (AIDS)

3316
Q

Etiology of AIDS

A

HIV1 - U.S. Central Africa, Europe, Asia

       - inferred origin: Common chimpanzees
       - global prevalende
       - mutated from simian immunodeficiency

HIV2 - West Africa

       - less virulence, less infectivity
       - inferred origin: Sooty mangabey
3317
Q

Risk group for HIV

A 
Homosexuals or bisexual males
IV users (25% chance)
Hemophiliacs
Blood transfusion recipients (90% chance)
Heterosexual contacts
3318
Q

Transmission of HIV

A 
Sexual contact (Dominant mode of infection)
Parenteral (IV drug needle, blood transfusion)
Vertical transmission (25% chance)
3319
Q

Route of vertical transmission

A

In utero via placental spread
During delivery via child birth
After birth via breastmilk

3320
Q

Two major target of HIV

A

Immune system and CNS

3321
Q

Immunologic alterations of HIV

A

Loss of CD4+ T cells (Dendritic cells and macrophages are infected)
Abnormalities of B-cell function

3322
Q

True or False.

Receptive intercourse causes an individual to be more predisposed to HIV infection than insertive.

A

True.
0.04-3% receptive anal intercourse
0.03% insertive anal intercourse
0.05-0.20% receptive penile-vaginal intercourse
0.01-0.35% insertive penile-vaginal intercourse
0-0.04% receptive oral intercourse
0-0.005% insertive oral intercourse

3323
Q

Modes of destruction of CD4+ T cells during HIV infections

A

Directly destroyed by virus
Subjected to apoptosis
Killed by cytotoxic T lymphocytes

3324
Q

Phases of HIV infection

A

Acute retroviral syndrome
Middle chronic phase
Final or crisis phase

3325
Q

2-4 weeks self-limited, acute flu-like illness

3-7 weeks post exposure, serum conversion of the virus

A

Acute retroviral phase

3326
Q

Asymptomatic or generalized lymphadenopathy

Continued viral replication

A

Middle chronic phase

3327
Q

Full blow AIDS
Presence of opportunistic infections
Lasting 7-10 years without chronic treatment

A

Final or crisis phase

3328
Q

Stage of HIV infection where patient is asymptomatic with acute retroviral syndrome

A

Primary HIV infection

3329
Q

Stage of HIV infection where patients are asymptomatic, CD4+ T cells >500 uL and persistent generalized lymphadenopathy

A

Clinical Stage 1

3330
Q

Stage of HIV infection where minor mucocutaneous manifestation in the URT are present with CD4+ T cells <500 uL

A

Clinical stage 2

3331
Q

Stage of HIV infection where weight loss, chronic diarrhea, persistent fever, oral candidiasis and other symptoms are more pronounced

A

Clinical Stage 3

3332
Q

Stage of HIV infection considered as full blown AIDS with the presence of indicator diseases such as Pneumocystis jirovecii pneumonia, Kaposi’s sarcoma (HIV8), candidiasis, and other opportunistic infection

A

Clinical Stage 4

3333
Q

Clinical features of AIDS

A

Young homosexual or IV drug abuser, positive HIV Ab test
Early and middle phase: Acute symptoms or generalized lymphadenopathy
Late: Fever, weight losee, generalized lymphadenopathy, Pneumocytosis carinii, Kaposi’s sarcoma, lymphoma, neurologic disease

3334
Q

Morphology of AIDS

A

Non-specific
Widespread opportunistic infection
Malignant neoplasms: Kaposi’s sarcoma, B-cell lymphomas, primary lymphoma of the brain, invasice cancer of the uterine cervic
Neurologic: aseptic meningitis, peripheral neuropathy, progressive encephalopathy (AIDS-dementia complex)
Lymph nodes: Non-Hodgkin’s lymphoma
Early: follicular hyperplasia (B-cell activation)
Late: follicular involution and generalized lymphocytic depletion

3335
Q

Prognosis of AIDS

A

Dismal:
Most progress to AIDS in 10 years of infection
No definitive treatment yet, only anti-retroviral therapy that contain HIV and maintain CD4+ T cell counts
Without treatment, a patient with AIDS will die in 1 year

3336
Q

Other causes of secondary immunodeficiency

A

Cancer chemotherapy
Involvement of bone marrow in metastasis
Protein-calorie malnutrition (Folate deficiency)
Removal of the spleen

3337
Q

Pathogenic fibrillar or misfolded proteins that accumulate within the tissues and organs

A

Amyloids

3338
Q

Group of diseases common of having deposition of amyloids

A

Amyloidosis

3339
Q

Aggregate into insoluble, cross-beta-pleated sheet tertiary conformation which will be deposited extracellularly causing pressure atrophy to adjacent parenchyma

A

Amyloidosis

3340
Q

Fibrillar deposits bind to _________

A

Proteoglycans
Glycosaminoglycans (heparan sulfate and dermatan sulfate)
Plasma proteins

3341
Q

Diagnosis of Amyloidosis

A 
Biopsy and characteristic congo red stain
Polarizing microscope (amyloid appears apple green birefringence)
3342
Q

Morphology of Amyloidosis in the kidney

A

Enlarged, pale gray, waxy

Chronic vascular occlusion ➡️ shrunken protracted organ in advance disease

3343
Q

Morphology of Amyloidosis in the spleen

A

Unapparent grossly

Sago spleen: tapioca like granules within splenic follicles

Lardaceous spleen: due to deposition in red pulp causing fusion of the deposits forming large geographic areas of amyloid

3344
Q

Morphology of Amyloidosis in the liver

A

Unapparent grossly
Hepatomegaly
Deposits in space of Dissse which cause pressure atrophy leading to hepatic replacement

3345
Q

Morphology of Amyloidosis in the heart

A

Subendocardial deposits

3346
Q

Clinical manifestation of Amyloidosis

A 
Non-specific
Renal involvement
Cardiac amyloidosis
GI amyloidosis
Vascular amyloidosis

Pathology (5 decks)

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