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MOD I Exam II > Chapter 6 > Flashcards

Chapter 6 Flashcards

1
Q

Component cell of innate immunity found in epithelia, lymphoid organs, and most tissues; captures protein Ags and displays peptides for recognition by T cells as well as stimulates secretion of cytokines

A

Dendritic cells

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2
Q

Component cell of innate immunity that functions in early protection against many viruses and intracellular bacteria

A

Natural killer cells

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3
Q

Component cells of innate immunity that look like lymphocytes but have features of innate immunity

A

Innate lymphoid cells

[lack TCRs but produce cytokines similar to T cells; function in early defense against infections, recognize and eliminate stressed cells, provide cytokines that influence differentiation of T cells]

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4
Q

Types of plasma proteins and their functions in innate immunity

A

Complement system - proteins that are activated by microbes using alternative and lectin pathways

Mannose-binding lectin and C-reactive protein - coat microbes and promote phagocytosis

Lung surfactant - provides protection against inhaled microbes

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5
Q

3 possible cellular locations for PRRs

A

PM receptors detect extracellular microbes (TLRs, MBLs)

Endosomal receptors detect ingested microbes (TLRs)

Cytosolic receptor detect microbes in cytoplasm (NLRs, RIG-like receptors)

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6
Q

Receptor type that recognizes a distinct type of microbial molecule before signaling a common pathway that leads to activation of transcription factors such as NF-kB and Interferon regulatory factors (IRFs)

A

Toll-like receptors (TLRs)

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7
Q

Function of NF-kB and IRFs activated by TLR binding

A

NF-kB - stimulates synthesis and secretion of cytokines and the expression of adhesion molecules critical for the recruitment and activation of leukocytes

Interferon Regulatory Factors (IRFs) - stimuates production of antiviral cytokines (type I interferons)

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8
Q

Cytosolic receptors that recognize a wide variety of substances and typically signal via the inflammasome

A

NLRs

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9
Q

What types of substances are recognized by NLRs?

A

Products of necrotic cells (e.g., uric acid and released ATP)

Ion disturbances (e.g., loss of K+)

Some microbial products

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10
Q

Result of gain-of-function mutaton in an NLR

A

Periodic fever syndrome, called autoinflammatory syndrome (can be treated with IL-1 antagonists)

[NLR may also play a role in gout, obesity-associated type 2 DM, atherosclerosis, etc.]

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11
Q

Activation of NLRP-3 signals via the inflammasome to activate caspase-1, which cleaves _____ to _______, which is secreted leading to acute inflammation and fever

A

Pro-IL-1B; IL-1B

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12
Q

Receptors found in the PM of macrophages and dendritic cells; function in detection of fungal glycans and elicit inflammatory reactions

A

C-type lectin receptors

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13
Q

Receptor type found in cytosol of most cell types; detects nucleic acids of viruses and stimulates production of antiviral cytokines

A

RIG-like receptors

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14
Q

Receptor type found on neutrophils, macrophages, and most leukocytes; recognizes bacterial peptides containing N-formylmethionyl

A

GPCRs

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15
Q

Receptor type responsible for recognizing microbial sugars leading to phagocytosis of the microbe

A

Mannose receptors

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16
Q

______ lymphocytes are those that have not yet encountered the Ag for which they are specific. Once they are activated by recognition of Ags, they either become _____ cells that eliminate microbes, or _____ cells that can react rapidly and strongly to that Ag in future encounters

A

Naive; effector; memory

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17
Q

Lymphocytes can respond to multiple Ags, but once exposed to one, a lymphocyte will undergo ____ _____

A

Clonal selection (all lymph with same specificity are clones)

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18
Q

Primary mechanism of developing lymphocyte diversity (when does it occur? What enzymes?)

A

Somatic recombination of the genes that encode the receptor proteins

Occurs during lymphocyte maturation (in thymus for Ts, in bone marrow for Bs)

Mediated by enzymes RAG-1 and RAG-2

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19
Q

Result of inherited defects in RAG-1 and RAG-2

A

Failure to generate mature lymphocytes

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20
Q

The majority of lymphocytes in blood and tissue are T lymphocytes. These recognize specific cell-bound Ag via a specific TCR. 95% of TCRs are heterodimers made up of an ___ and ____ polypeptide chain

These receptors recognize Ags presented by _____ molecules on the surfaces of APCs, a restriction which ensures T cells only see cell-associated Ags

A

Alpha; beta

MHC

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21
Q

A small population of mature T cells are gamma-delta T cells. What do these recognize and where are they found?

A

Recognizes peptides, lipids, and small molecules, without assistance from MHC proteins

They aggregate at epithelial surfaces (e.g., skin, GI, urogenital tracts)

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22
Q

A small subset of NK-T cells express a very limited diversity of TCRs. They recognize _______ that are displayed by the MHC-like molecule _______

A

Glycolipids; CD1

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23
Q

Signal 1 consists of CD4+ T cell binding class II MHC of APC via alpha and beta chain of the TCR. What interaction provides signal 2?

A

CD28 on T cell binds CD80/CD86 on APC

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24
Q

B cells recognize Ag via the B cell Ag receptor complex

Membrane-bound Abs of the ____ and ___ isotypes are present on the surface of all mature naive B cells. After stimulation by Ag they develop into ___ cells

A

IgM; IgD; plasma

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25
Q

Besides BCR, what are 2 other molecules essential for signaling B lymphocytes?

A

Type 2 complement receptor (CR2, or CD21)

CD40 - receives signals from helper T cells

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26
Q

The type 2 complement receptor (CR2, or CD21) that is essential for BCR signaling is also used in what pathologic mechanism?

A

CR2 is used by EBV to enter and infect B cells

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27
Q

What are langerhans cells

A

Dendritic cells located in the skin

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28
Q

Location and function of follicular dendritic cells

A

Located in germinal centers of lymphoid follicles in the spleen and lymph nodes

Trap Ags bound to Abs or complement proteins

Present Ags to B cells and select the highest affinity B cells

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29
Q

____ cells can activate macrophages and enhance their ability to kill ingested microbes

Macrophages phagocytose and destroy microbes that are opsonized by ___ or _____

A

T

IgG; C3b

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30
Q

Primary function is to destroy irreversibly stressed and abnormal cells, and do so without prior exposure to or activation by these cells

A

NK cells

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31
Q

Surface molecules of NK cells

A

CD16 — an Fc receptor for IgG (lyse IgG-coated target cells in ADCC)

CD56 - function unknown

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32
Q

NK cells are regulated by a balance between activating and inhibitory receptors and by many cytokines

___ and ___ stimulate proliferation

___ activates killing and secretion of _____, which serves to activate macrophages to destroy ingested microbes

A

IL-2; IL-15

IL-12; IFN-y

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33
Q

A virally infected cell will no longer be expressing ______ which is considered an inhibitory receptor for NK cells. Thus when the cell is not virally infected, the inhibitory receptor is engaged and the cell is not killed

A

Class I MHC

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34
Q

Genes that encode HLA molecules are clustered on chromosome _____; they are highly polymorphic with thousands of alleles of MHC genes

A

6

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35
Q

Where are MHC class I expressed and what are the types, what type of peptides do they display?

A

Expressed on all nucleated cells and platelets

HLA-A
HLA-B
HLA-C

Display peptides derived from proteins located in the cytoplasm (e.g., viral and tumor Ags)

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36
Q

Where are MHC class II expressed, where are they encoded, and what do they display

A

Mainly expressed on macrophages, B cells, and dendritic cells

Encoded by HLA-D:
HLA-DP
HLA-DQ
HLA-DR

Display Ags that are internalized into vesicles; typically from extracellular microbes and soluble proteins

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37
Q

How are HLA molecules inherited?

A

Individuals inherit one set of HLA genes from each parent

Typically 2 different molecules for every locus

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38
Q

Cytokines associated with innate immune responses

A

Produced by macrophages, dendritic cells, NK cells, etc.

Induce inflammation and inhibit viral replication

TNF, IL-1, IL-12, Type I IFNs, IFN-y, and chemokines

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39
Q

Cytokines role in adaptive immune responses

A

Produced mainly by CD4+ T cells

Promote lymphocyte proliferation and differentiation and to activate effector cells

IL-2, IL-4, IL-5, IL-17, and IFN-y

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40
Q

Role of cytokines in hematopoiesis

A

Stimulate hematopoeisis via colony-stimulating factors — role is to increase leukocyte numbers

GM-CSF; IL-7

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41
Q

The ______ serves the same role in immune responses to ______ Ags as lymph nodes do in response to lymph-borne Ags. Blood entering this organ flows through a network of ______ where Ags are trapped by _____ and _____

A

Spleen; bloodborne; sinusoids; DCs; macrophages

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42
Q

Major cytokines produced; immunological reactions triggered; host defense against; and role in disease of Th1 CD4+ cells

A

Cytokines: IFN-y

Reactions: macrophage activation, stimulation of IgG production

Host defense against IC microbes

Role in autoimmune and other chronic inflammatory diseases like IBD, psoriasis, and granulomatous inflammation

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43
Q

Major cytokines produced; immunological reactions triggered; host defense against; and role in disease of Th2 CD4+ cells

A

Cytokines: IL-4, IL-5, IL-13

Reactions: stimulation of IgE production; activation of mast cells and eosinophils

Host defense against helminthic parasites

Role in allergies

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44
Q

Major cytokines produced; immunological reactions triggered; host defense against; and role in disease of Th17 CD4+ cells

A

Cytokines: IL-17; IL-22

Reactions: recruitment of neutrophils, monocytes

Host defense against EC bacteria; fungi

Role in autoimmune and other chronic diseases like IBD, psoriasis, MS

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45
Q

Functions of Abs

A

Neutralization of microbe and toxins

Opsonization and phagocytosis

ADCC

Complement activation leading to lysis of microbes, phagocytosis of opsonized microbes, and inflammation

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46
Q

Immune mechanism of type I hypersensitivity

A

[immediate hypersensitivity]

Production of IgE —> immediate release of vasoactive amines and other mediators from mast cells; later recruitment of inflammatory cells

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47
Q

Histopathologic lesions associated with type I hypersensitivities

A 
Vascular dilation
Edema
Smooth muscle contraction
Mucus production
Tissue injury
Inflammation
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48
Q

Prototypical disorders associated with type I hypersensitivities

A

Anaphylaxis
Allergies
Bronchial asthma (atopic forms)

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49
Q

Immune mechanism associated with type II hypersensitivities

A

[Ab-mediated hypersensitivity]

Production of IgG, IgM —> binds to Ag on target cell or tissue —> phagocytosis or lysis of target cell by activated complement or Fc receptors; recruitment of leukocytes

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50
Q

Histopathologic lesions associated with type II hypersensitivities

A

Phagocytosis and lysis of cells; in some diseases, funcntional derangements without cell or tissue injury

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51
Q

Prototypical disorders associated with type II hypersensitivities

A

Autoimmune hemolytic anemia

Goodpasture syndrome

[also autoimmune thrombocytopenic purpura, pemphigus vulgaris, vasculitis caused by ANCA, acute rheumatic fever, myasthenia gravis, graves disease, insulin-resistant diabetes, pernicious anemia]

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52
Q

Immune mechanisms associated with type III hypersensitivities

A

[immune complex-mediated]

Deposition of Ag-Ab complexes —> complement activation —> recruitment of leukocytes by complement products and Fc receptors —> release of enzymes and other toxic molecules

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53
Q

Histopathologic lesions associated with type III hypersensitivity

A 
Inflammation
Necrotizing vasculitis (fibrinoid necrosis)
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54
Q

Prototypical disorders associated with type III hypersensitivities

A

SLE
Poststreptococcal glomerulonephritis
Serum sickness
Arthus reaction

[also polyarteritis nodosa and reactive arthritis; most result in nephritis, joint pain, systemic vasculitis, etc.]

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55
Q

Immune mechanisms of type IV hypersensitivity

A

[cell-mediated hypesensitivity]

Activated T cells —> release of cytokines, inflammation, and macrophage activation; T cell mediated cytotoxicity

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56
Q

Histopathologic lesions associated with type IV hypersensitivity

A

Perivascular infiltrates, edema, granuloma formation; cell destruction

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57
Q

Prototypical disorders associated with type IV hypersensitivity

A

Contact dermatitis
Multiple sclerosis
Type I DM
Tuberculosis

[also rheumatoid arthritis, IBD, psoriasis]

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58
Q

Immediate vs. late phase reaction with type I hypersensitivity

A

Immediate reaction: vasodilation, vascular leakage, smooth muscle spasm or glandular secretion — occurs minutes after exposure and subsides w/i a few hours

Late-phase (e.g., allergic rhinitis and bronchial asthma): 2-24 hours after initial exposure and lasts several days. Infiltration with eosinophils, neutrophils, basophils, monocytes, and CD4+

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59
Q

Describe activation of mast cells

A

Activated by cross-linking of high-affinity IgE Fc receptors

IgE coated mast cells are “sensitized” — sensitive to second encounter with specific Ag

[mast cells also triggered by complement components C5a and C3a, chemokines, drugs like codeine and morphine, adenosine, melittin aka bee venom, physical stim like heat/cold/sun]

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60
Q

What subset of CD4+ T cells plays an important role in Type I hypersensitivity?

A

Th2 produces important cytokines

IL-4 stimulates class switching to IgE production + more production of Th2 cells

IL-5 induces development and activation of eosinophils

IL-13 enhances IgE production and acts on epithelial cells to stimulate mucus secretion

Also chemokine production

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61
Q

During the late phase rxn of type I hypersensitivity, leukocytes — especially eosinophils — are recruited which amplify and sustain the inflammatory response without Ag present. Eosinophils damage tissue via release of what substances?

A

Proteolytic enzymes
Major basic protein
Eosinophil cationic protein

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62
Q

___ =increased propensity to develop immediate hypersensitivity reactions

A

Atopy

[have higher serum IgE levels and more IL-4 producing Th2 cells]

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63
Q

Non-atopic allergy is responsible for 20-30% of immediate hypersensitivity reactions. What are the triggers for these types of reactions?

A

Triggered by non-antigenic stimuli like temp extremes and exercise

Does not involve Th2 cells or IgE

Likely d/t mast cells that are abnormally sensitive to activation

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64
Q

Examples of Ab-mediated cell destruction and phagocytosis in disease

A

Transfusion reactions

Hemolytic disease of newborn

Autoimmune hemolytic anemia, agranulocytosis, and thrombocytopenia

Certain drug reactions where drug acts as hapten attaching to PM of RBCs

Myasthenia gravis

Graves disease

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65
Q

Mechanism of Ab-mediated cell dysfunction in myasthenia gravis

A

Abs react with ACh receptors in motor end plates of skeletal muscles and block neuromuscular transmission —> muscle weakness

66
Q

Mechanism of Ab-mediated cell dysfunction in graves disease

A

Abs against TSH receptors on thyroid epithelial cells stimulate the cells —> hyperthyroidism

67
Q

Decreased serum levels of C3 can be used to detect what type of hypersensitivity?

A

Type III

68
Q

What 2 subsets of CD4 cells play an important role in type IV hypersensitivities?

A

Th1 — secrete IFN-y to activate macrophages

Th17 — secrete IL-17, IL-22, chemokines, etc. to recruit neutrophils and monocytes; also produce IL-21 to amplify Th17 response

69
Q

The tuberculin reaction is an example of what type of hypersensitivity response?

A

Type IV

70
Q

Mechanism of central tolerance in T cells

A

Negative selection or deletion — via apoptosis

Some may develop into Treg cells

71
Q

Mechanism of central tolerance for B cells

A

Many will undergo receptor-editing, otherwise undergo apoptosis

72
Q

Mechanisms of peripheral tolerance

A

Anergy
Suppression by Treg cells
Deletion/apoptosis

73
Q

Immune-privileged sites (no communication with blood and lymph)

A

Testis
Eye
Brain

74
Q

Diseases associated with DRB1 HLA alleles

A

Rheumatoid arthritis (anti-CCP Ab positive)

Type I DM

Multiple sclerosis

SLE

75
Q

Disease associated with B*27 HLA allele

A

Ankylosing spondylitis

76
Q

Disease associated with DQA1 HLA allele

A

Celiac disease

77
Q

Diseases associated with PTPN22

A

RA
T1D
IBD

78
Q

Diseases associated with IL23R

A

IBD
PS
AS

79
Q

Diseases associated with CTLA4

A

T1D

RA

80
Q

Diseases associated with IL2RA

A

MS

T1D

81
Q

Diseases associated with NOD2 gene

A

IBD

82
Q

Diseases associated with ATG16 gene

A

IBD

83
Q

Disease associated with IRF5; IFIH1 genes

A

SLE

84
Q

Classic example of molecular mimicry

A

Rheumatic heart disease — Ab against streptococcal proteins cross-react with myocardial proteins and cause myocarditis

85
Q

In what way might infections protect against some autoimmune diseases?

A

Infections promote low-level IL-2 production, and this is essential for maintaining Treg cell population

86
Q

Disease affecting african american+hispanics more than whites, involves multiple organs like skin, joints, kidney, and serosal membranes, and injury is caused mainly by deposition of immune complexes and binding of Abs to various cells and tissues

A

SLE

87
Q

Criteria for SLE

A 
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis
Renal disorder
Neurologic disorder
Hematologic disorder
Immunologic disorder
Antinuclear Ab

[greater than 4 criteria = SLE]

88
Q

All systemic autoimmune diseases typically have what type of autoantibodies?

A

Antinuclear antibodies (ANAs)

89
Q

Examples of systemic autoimmune diseases other than SLE

A

Systemic sclerosis
Sjogren syndrome
Autoimmune myositis
Rheumatoid arthritis

90
Q

Antinuclear Abs are directed against nuclear Ags. What are the 4 categories?

A

Abs to DNA
Abs to histones
Abs to nonhistone proteins bound to RNA
Abs to nucleolar Ags

91
Q

Antibodies to _______ and ______ are virtually diagnostic of SLE

A

dsDNA; Smith (Sm) antigen

92
Q

6 patterns of glomerular disease associated with SLE; note which is most common vs. least common

A

Minimal mesangial lupus nephritis (least common)

Mesangial proliferative lupus nephritis

Focal lupus nephritis

Diffuse lupus nephritis (MOST COMMON/MOST SEVERE)

Membranous lupus nephritis

Advanced sclerosing lupus nephritis

[these are listed in order from class I —> class VI]

93
Q

Rarely, _________ _____ may be the dominant abnormality in glomerular disease associated with SLE

A

Tubulointerstitial lesions

94
Q

Describe skin changes associated with SLE

A

Characteristic butterfly rash

Can have similar rash on extremities and trunk; exposure to sunlight incites or accentuates the erythema

Immunoflourescence: deposition of Ig and complement along dermal-epidermal junction — can be present in uninvolved skin (also seen in scleroderma or dermatomyositis)

95
Q

SLE effects on CV system

A

Any layer of heart can be damaged

Myocarditis can lead to tachycardia and ECG abnormalities

CAD can lead to angina, MI

Valvular abnormalities (mitral and aortic valves) can lead to stenosis and/or regurg

Valvular (aka Libman Sacks) endocarditis — uncommon now d/t steroid tx, aka nonbacterial verrucous endocarditis

96
Q

CNS involvement with SLE

A

Neuropsychiatric symptoms d/t non-inflammatory occlusion of small vessels; may be d/t endothelial damage by autoantibodies or immune complexes

97
Q

Spleen involvement with SLE

A

Splenomegaly
Capsular thickening
Follicular hyperplasia

98
Q

Lung involvement with SLE

A

Pleuritis and pleural effusions
Chronic interstitial fibrosis
Secondary pulmonary HTN

99
Q

SLE effect on blood vessels and joints

A

Blood vessels — acute necrotizing vasculitis involving capillaries, small arteries, and arterioles

Joints — non-erosive synovitis with little deformity (opposite of RA)

100
Q

Subtype of lupus causing primarily skin manifestations with plaques showing varying degrees of edema, erythema, scaliness, follicular plugging, and skin atrophy surrounded by an elevated erythematous border

A

Chronic discoid lupus erythematosus

101
Q

Subtype of lupus presenting with predominant skin involvement in which skin rash tends to be widespread, superficial, and non-scarring; most patients have mild systemic symptoms

A

Subacute cutaneous lupus erythematosus

102
Q

Subset of lupus manifesting as a LE-like syndrome in patients receiving hydralazine, procainamide, isoniazid, D-penicillamine, or anti-TNF therapy — affects multiple organs but renal and CNS involvement is uncommon

A

Drug-induced lupus erythematosus

103
Q

Chronic disease characterized by keratoconjunctivitis sicca and xerostomia

A

Sjogren syndrome

104
Q

Biopsy of the lip is required for dx of Sjogrens. What are the intense lymphocytic responses in the tissues involved? (Early vs over time)

A

Early: mixture of polyclonal T and B cells

Over time: a dominant B-cell clone can emerge —> marginal zone lymphoma

105
Q

Disease characterized by chronic inflammation thought to be result of autoimmunity leading to widespread damage to small blood vessels as well as progressive interstitial and perivascular fibrosis in skin and multiple organs

A

Systemic sclerosis (scleroderma)

106
Q

Majority of scleroderma pts progress with death from what potential complications?

A

Renal failure
Cardiac failure
Pulmonary insufficiency
Malabsorption

107
Q

Clinical features of scleroderma

A

Mostly females 50-60

Skin thickening

Raynaud phenomenon (episodic vasoconstriction of arteries and arterioles of extremities)

Dysphagia from esophageal fibrosis

Respiratory difficulty

Myocardial fibrosis —> arrhythmias or cardiac failure

Renal: mild proteinuria, malignant HTN

108
Q

Some patients with limited scleroderma have what is known as CREST syndrome. What is the combination of symptoms associated with this syndrome?

A 
Calcinosis
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia
109
Q

Disease with clinical features that are a mixture of the features of SLE, systemic sclerosis, and polymyositis

A

Mixed CT disease (not a distinct entity, can evolve into classic SLE or scleroderma)

110
Q

Serious complications of mixed CT disease

A

Pulmonary HTN
Interstitial lung disease
Renal disease

111
Q

Relatively new disease most often affecting middle-aged and older men

First characterized in autoimmune pancreatitis and now reported in virtually all organs

Characterized by tissues infiltrated with Ab-producing plasma cells and lymphocytes (mainly T cells), storiform fibrosis and obliterative phlebitis, and usually increased serum Ab levels

A

IgG4 related disease

112
Q

Recognition of alloantigens in kidney graft via direct vs. indirect path

A

Direct: donor APC in graft recognized by host T cells; CD4+ secrete IFN-y to activate macrophages, CD8+ cause endothelitis in renal blood vessels and damge renal tubule cells

Indirect: recipients APC recognizes graft cells and presents ingested peptides to CD4+ cells which secrete IFN-y, activating macrophages and damaging renal blood vessels. CD4+ also cause differentiation of B cells into plasma cells which secrete Abs that cause endothelial injury in renal blood vessels

113
Q

Acute vs. chronic T-cell mediated rejection of organs

A

Acute: within months, associated with clinical and biochemical signs of organ failure, cytokine secretion by CD4s, increased vascular permeability and local accumulation of lymphocytes and macrophages

Chronic: lymphocytes react against alloantigens in vessel wall; secrete cytokines that induce local inflammation

114
Q

Ab-mediated reactions to graft tissue

A

Hyperacute: occurs when PREFORMED antidonor Abs are present in recipient (d/t previous transplant, prior transfusion, multiparous women)

Acute: antidonor Abs produced after transplant, initial target seems to be graft vasculature

Chronic: insidious development, primarily affects vasculature

115
Q

HLA matching is not typically done in what types of organ transplants?

A

Liver
Heart
Lungs

116
Q

Types of immunosuppressive therapy

A

Steroids - reduce inflammation

Mycophenolate mofetil - inhibits lymphocyte prolif

Tacrolimus (FK506) - inhibits T cell function

117
Q

___ virus can reactivate, infect renal tubules and may even cause graft failure; complication of immunosuppression

A

Polyoma virus

118
Q

Immunosuppression carries risk of re-activation of what latent viruses?

A

EBV-induced lymphomas

HPV-induced squamous cell carcinomas

Kaposi sarcoma (HHV8)

119
Q

What are hematopoietic stem cell (bone marrow) transplants used for?

A

Hematologic malignancies

Bone marow failure syndromes like aplastic anemia

Inherited stem cell defects like sickle cell anemia, thalassemia, and immunodeficiency states

120
Q

Disease that occurs when immunologically competent cells or their precursors are transplanted into immunologically crippled recipients

A

Graft-vs-Host disease (GVHD)

The transferred cells recognize alloantigens in host and attack host tissues

Seen MOST COMMONLY in setting of HSC transplantation; rarely occurs in transplant of solid organs or transfusions of un-irradiated blood

121
Q

GVHD is mediated by T cells contained in transplanted donor cells, so the disease can be eliminated by depleting donor T cells prior to transfusion. This can have what complications?

A

Recurrence of tumor in leukemic patients

Increased incidence of graft failures

Increased rates of EBV-related B cell lymphoma

122
Q

Inherited defects in innate immunity

A

LAD 1 (mutations in beta chain of integrins)
LAD2 (mutations in selectin receptor)
Chediak-Higashi (mutations affecting lysosome membrane traffic)
CGD (decreased oxidative burst)
MPO deficiency (decreased microbial killing)

C2,C4 def (defective classic path)
C3 def (all complement functions defective)
Complement regulatory protein def (excess activation, angioedema, paroxysmal hemoglobinuria)
123
Q

Defects in both humoral and cell mediated responses in which infants present with thrush, diaper rash, FTT, GVHD rash, and recurrent infections

A

SCID (usually x linked, also can be AR)

124
Q

Failure of B cell precursors to develop into mature B cells, serum levels of all classes of Ig are depressed and plasma cells are absent throughout the body

A

X-linked agammaglobulinemia (bruton)

125
Q

X-linked agammaglobulinemia puts patients at risk for infections that rely on Abs to clear them — what are some examples?

A

Recurrent bacterial infections of respiratory tract — H.influenzae, Strep pneumoniae, Staph aureus

Viruses in bloodstream or mucosal secretions — enteroviruses like echovirus, poliovirus, and coxsackivirus

Giardia lamblia (intestinal protozoan)

126
Q

Etiology of DiGeorge syndrome and the consequences

A

Failure of development of 3rd and 4th pharyngeal pouches which usually give rise to thymus, parathyroids, C cells of thyroid, ultimobranchial body

Leads to loss of T cell mediated immunity (poor defense against fungal and viral infections), hypocalcemia induced tetany, and congenital defects of heart and great vessels, abnormal appearance of mouth, ears, facies

Component of 22q11 deletion syndrome

127
Q

Disorder caused by defect in ability of helper T cells to deliver activating signals to B cells and macrophages, typically X-linked, and presents with recurrent pyogenic infections and/or pneumocystis jiroveci pneumonia

A

Hyper-IgM syndrome

128
Q

Group of disorders with hypogammaglobulinemia that can be sporadic or inherited form. Patients have normal/near-normal level of B cells but they aren’t ablel to differentiate into plasma cells. Clinically resembles X-linked agammaglobulinemia but M=F

A

Common variable immunodeficiency (CVID)

129
Q

Disease that can be familial or acquired in association with toxoplasmosis, measles, or other viral infection. Most patients are asymptomatic but those who have symptoms tend to have recurrent sinopulmonary infections and diarrhea as well as high frequency of respiratory tract allergy and autoimmune diseases like SLE, RA, and celiac

A

Isolated IgA deficiency — extremely low levels of both serum and secretory IgA

130
Q

X-linked immunodeficiency characterized by thrombocytopenia, eczema, and recurrent infections leading to early death if not treated with HSC transplant

A

Wiskott aldrich syndrome

131
Q

Autosomal recessive immunodeficiency characterized by abnormal gait, vascular malformations, neurologic deficit, increased incidence of tumors, and immunodeficiency

A

Ataxia telangiectasia

132
Q

Modes of HIV transmission

A

Sexual transmission
Parenteral transmission
Mother-to-infant

133
Q

Mechanisms of parenteral transmission of HIV

A

IV drug abusers (needle sharing)

Hemophiliacs who received factor VIII and IX concentrates

Recipients of blood transfusions

134
Q

Mechanisms of mother-to-infant transmission of HIV

A

In utero by transplacental spread

During delivery through infected birth canal

After birth by ingestion of breast milk

135
Q

HIV first infects which cells? What do these cells have in common?

A

T cells
DCs
Macrophages

[all have CD4]

136
Q

Major abnormalities in immune function in AIDS

A

Lymphopenia
Decreased T cell function in vitro and in vivo
Polyclonal B cell activation
Altered monocyte/macrophage functions

137
Q

HIV 1 can infect and multiply in terminally non-dividing macrophages, dependent on ____ gene

A

Vpr

138
Q

DCs role in HIV

A

Mucosal DCs — infected and transport to regional LNs then transmit to CD4 T cells. Lectin like receptor specifically binds HIV and displays it in intact infectious form to T cells

Follicular DCs in LNs — potential reservoirs, most virus particles found on their dendritic processes, trap virions coated with anti-HIV Abs thus retaining ability to infect CD4+ cells

139
Q

B cells and HIV

A

Polyclonal activation of B cells —> GC hyperplasia, bone marrow plasmacytosis, hypergammaglobulinemia, formation of circulating immune complexes

Can’t mount Ab responses to newly encountered Ag

140
Q

HIV and the brain

A

Nervous system is major target; HIV infects macrophages and microglial cells

141
Q

HIV shows few or no clinical manifestations during clinical latency period. What are some examples?

A

Thrush, vaginal candidiasis, herpes zoster, mycobacterial Tb, autoimmune thrombocytopenia

142
Q

How does HIV evade immune detection

A

Destroys CD4 cells critical for effective immunity

Antigenic variation

Down-modulation of Class I MHCs on infected cells so viral Ags aren’t recognized by CD8

May also evolve and switch from relying solely on CCR5 to enter target cell to relying on either CXCR4 or both

143
Q

Clinical features of AIDS

A 
Fever and weight loss
Diarrhea
Generalized lymphadenopathy
Neurologic disease
Multiple opportunistic infections
Secondary neoplasms
144
Q

Tx for HIV and potential downsides

A

HAART — highly active antiretroviral therapy

Downsides = immune reconstitution inflammatory syndrome, adverse side effects like lipoatrophy, lipoaccumulation, elevated lipids, insulin resistance, peripheral neuropathy, permature CV, kidney, and liver disease

145
Q

Extracellular deposits of fibrillar proteins leading to tissue damage and functional compromise

A

Amyloidosis

146
Q

AIDS-defining protozoal and helminthic infections

A

Cryptosporidiosis or isosporidiosis (enteritis)

Pneumocystosis (pneumonia or disseminated infection)

Toxoplasmosis (pneumonia or CNS infection)

147
Q

AIDS-defining fungal infections

A

Candidiases (esophageal, tracheal, pulmonary)

Cryptococcosis (CNS infection)

Coccidioidomycosis (disseminated)

Histoplasmosis (disseminated)

Pneumocystis jiroveci

148
Q

AIDS-defining bacterial infections

A

Mycobacteriosis (atypical, e.g., mycobacterium avium-intracellulare, disseminated or extrapulmonary; mycobacterium tuberculosis)

Nocardiosis (pneumonia, meningitis, disseminated)

Salmonella infections, disseminated

149
Q

AIDS-defining viral infections

A

CMV (pulmonary, intestinal, retinitis, or CNS)

HSV (localized or disseminated)

Varicella-zoster virus (localized or disseminated)

JC virus — progressive multifocla leukoencephalopathy

150
Q

AIDS-defining neoplasms

A

Kaposi sarcoma (HHV8)
Primary lymphoma of brain (HBV)
Invasive cancer of uterine cervix or anus (HPV)

151
Q

Ig light chains are known as ________ proteins when found in the urine

A

“Bence Jones”

152
Q

Amyloidosis may occur due to production of abnormal amounts of protein d/t:

—Acquired mutations in Ig light chains which undergo limited proteolysis to form ____ proteins which deposit in tissues

—Chronic inflammation, macrophage activation, IL-1 and IL-6 release causing release of SAA protein from the liver, which undergoes limited proteolysis to form ____ proteins which deposit in tissues

A

AL

AA

153
Q

Amyloidosis may also occur d/t production of normal amounts of mutant protein such as mutant transthyretin, which aggregates to form deposits of ____ protein

What diseases would this be associated with?

A

ATTR

Familial amyloidotic neuropathies (several types)

Systemic senile amyloidosis

154
Q

Associated diseases, major fibril protein, and chemically related precursor protein in immunocyte dyscrasias with amyloidosis (primary amyloidosis)

A

Multiple myeloma and other monoclonal plasma cell proliferations

Major protein = AL

Precursor = Ig light chains, chiefly gamma type

155
Q

Associated diseases, major fibril protein, and chemically related precursor protein in reactive systemic amyloidosis (secondary amyloidosis)

A

Chronic inflammatory conditions

Major protein = AA

Precursor = SAA

156
Q

Associated diseases, major fibril protein, and chemically related precursor protein in hemodialysis-associated amyloidosis

A

Chronic renal failure

Major protein = AB2m

Precursor = B2-microglobulin (component of MHC molecules)

157
Q

Associated diseases, major fibril protein, and chemically related precursor protein in senile cerebral amyloidosis

A

Alzheimer disease

Major protein = AB

Precursor = APP

158
Q

Major fibril protein and chemically related precursor protein involved in familial mediterranean fever

A

AA

Precursor = SAA

159
Q

General structural effect amyloid has on organs

A

Progressive accumulation —> encroachment and pressure atrophy of adjacent cells

160
Q

Organ involvement in amyloidosis

A

Secondary to chronic inflamm: kidneys, liver, spleen, LNs, adrenals, thyroid

Amyloidosis associated with plasma cell proliferations: heart, GI tract, respiratory tract, peripheral nerves, skin, and tongue (but not specific)

161
Q

Clinical features of amyloidosis

A

Incidental finding with no clinical manifestations

Cause serious clinical problems and even death

Symptoms depend on magnitude of deposits and sites or organs affected

Initial clinical manifestations are nonspecific — weakness, weight loss, light-headedness, or syncope

Prognosis for generalized amyloidosis is poor

MOD I Exam II (3 decks)

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