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MOD Exam 2 > Diseases of the Immune System > Flashcards

Diseases of the Immune System Flashcards

1
Q

TLRs, NOD-like receptors/inflammasome, C-type Lectin Receptors are examples of what?

A

Pattern recognition receptors

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2
Q

Activation of TLRs leads to the synthesis and secretion of what?

A

NFkB which => cytokines and adhesion molecules

Interferon regulatory factors (IRFs) => antiviral cytokines

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3
Q

What receptors send signals through the inflammasome? What does this action result in?

A

NOD-like receptors

Inflammasome activates caspase-1 => IL-1 into biologically active form (GOF will cause periodic fever syndrome)

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4
Q

Where are the C-Type lectin receptors located? What do they detect?

A

PM of macrophages and DCs

Detect Fungal glycan => inflammatory rxn to fungi

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5
Q

What complement effectors function to cause inflammation?

A

C5a, C3a

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6
Q

What complement effectors function to cause phagocytosis?

A

C3b

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7
Q

What tissues serve as the location for lymphocyte stem cell generation and B-cell maturation?

A

Bone Marrow

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8
Q

What tissues serve as the location where lymphocytes interact with APCs and antigen in circulating lymph?

A

Lymph nodes

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9
Q

What tissues serve as the location where lymphocytes interact with blood-born Ag?

A

Spleen

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10
Q

Where are Hassall corpuscles located? What are they composed of?

A

Medulla of thymus

Squamous cell nests

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11
Q

The medulla of the thymus is where the maturing T-Cells interact with what?

A

Dendritic APCs with high levels of MHC I and II

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12
Q

What part of the peripheral lymphoid organs do the T cells predominate?

A

Paracortex

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13
Q

What part of the peripheral lymphoid organs do the B cells predominate?

A

Germinal centers

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14
Q

What are the actions of the Helper T Cells (CD4+)

A

Activation of macrophages

Promote proliferation and differentiation of T and B lymphocytes

Inflammation

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15
Q

What MHC class is located on all nucleated cells and recognize intracellular Ag? What T cells does it present to?

A

MHC Class I

CD8+

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16
Q

Where is MHC Class II typically located? What type of Ag do they recognize? What T Cells does it present to?

A

Antigen presenting cells

Recognize extracellular Ag (bacterial, allergens)

CD4+

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17
Q

What is the process by which MHC Class I presents to T cells?

A
  1. Ag recognizes intracell Ag
  2. Proteosome processes Ag into peptides
  3. Transport to ER, load into groove of MHC
  4. MHC-peptide complex presented to CD8+ (cytotoxic) T cells
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18
Q

What is the process by which MHC Class II presents to T cells?

A
  1. Extracell Ag recognized
  2. Endolysosomal enzymes process Ag into peptides
  3. Vesicles form w/ peptide and MHC II
  4. Complex presented to CD4+ (helper) T cells
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19
Q

Which chromosome encodes the HLA molecular structure for a given individual?

A

Chromosome 6

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20
Q

What HLA molecules are transcribed from the Class I section of chromosome 6?

A

HLA-A

HLA-B

HLA-C

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21
Q

What HLA molecules are transcribed from the Class II section of chromosome 6?

A

HLA-DP

HLA-DQ

HLA-DR

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22
Q

What is the T-Cell response during Cell-mediated immunity?

A

After presentation of the Ag, T cells go through:

Proliferation

Differentiation
Migration

Killing

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23
Q

Which Ab is the first to produced and the biggest?

A

IgM (pentamer)

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24
Q

Which Ab has the longest half-life?

A

IgG

(important in fetal protection)

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25
Q

What happens to the fetus if mom has an infection involving IgM late in her pregnancy?

A

Mom will be able to protect herself but fetus will have no protection because IgM is not passed to newborn

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26
Q

What Ab is involved in mucosal defense and present in high levels in the colostrum?

A

IgA

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27
Q

What Ab has the shortest half life? What receptor does it bind to on mast cells, basophils, and eosinophils?

A

IgE

High affinity binding to FC receptors

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28
Q

What immune response are NK cells involved in? How are they inhibited?

A

Innate immunity

Inhibited w/ MHC Class I expression and self MHC molecules

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29
Q

What receptor on NK cells recognize damaged cells and activate the NK cells?

A

NKG2D receptors

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30
Q

In a Type I Hypersensitivity rxn, what Ab do B cells switch to? What receptor does this Ab bind to?

A

IgE

IgE binds onto FcɛRI receptor

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31
Q

What interleukins are involved in a Type I Hypersensitivity reaction? What are their functions?

A

IL-4 - Class switching

IL-5 - Eosinophil activation

IL-13 - Enhanced IgE production

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32
Q

What mast cell mediators are involved in the immediate hypersensitivity reaction?

A

Degranulation - Histamine

Lipid mediators: Leukotrienes B4, C4, D4; Prostaglandin D2; Platelet Activating Factor

Involved in vasodilation, vascular leakage, smooth muscle spasm

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33
Q

What mediators are involved in the late phase hypersensitivity reaction? What are their functions?

A

Cytokines/chemokines - Leukocyte recruitment

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34
Q

Allergies and allergic reactions such as hives, angioedema, and anaphylaxis are all examples of what kind of hypersensitivity reaction?

A

Type I hypersensitivity

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35
Q

What leukocytes are recruited in the late phase of the Type I hypersensitivity reaction?

A

Inflammatory cells: eosinophils, basophils, neutrophils

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36
Q

An H&E stain shows a slide of epithelial cells with a dense infiltrate of inflammatory cells with red cells inside. The pt it was taken from has recurrent dysphagia and subsequent weight loss which has been ongoing since childhood. What is this disease?

A

Eosinophilic esophagitis

(Food Ag-driven disease of childhood, can’t swallow effectively/hurts to swallow)

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37
Q

What constitutes a Type II hypersensitivity rxn?

A

Reactions where Ab directly react with Ag present on the cell surface or ECM

Caused by AutoAb or exogenous Ag bound to cell surfaces

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38
Q

What Type II Hypersensitivity diseases are caused by opsonization and phagocytosis? What Ag is targeted?

A

Autoimmune hemolytic anemia - target red cell membrane ptns (Rh blood group Ag, I Ag)

Autoimmune thrombocytopenic purpura - target platelet membrane ptns

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39
Q

What is the basic mechanism of Type II Hypersensitivity via opsonization and phagocytosis?

A

Opsonized cell is recognized by the Fc receptor on the phagocyte and is devoured and lysed

Cell is gone => anemia, thrombocytopenia

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40
Q

What is the basic mechanism of the Type II Hypersensitivity reaction via Complement and Fc receptor-mediated inflammation?

A

Fc receptor recognizes and binds to Ag in basement membrane and deposit Ab

Complement is activated => Neutrophil enzymes, ROS released into tissues => inflammation and tissue injury

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41
Q

What Type II Hypersensitivity reactions are caused by complement and Fc receptor-mediated inflammation? What are their target Ag?

A

Vasculitis caused by ANCA - target neutrophil granule ptns

Goodpasture syndrome - target noncollagenous ptn in basement membrane of kidney glomeruli and lung alveoli

Acute rheumatic fever - streptococcal cell wall Ag, Ab cross-reacts with myocardial Ag

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42
Q

What is the basic mechanism of Type II Hypersensitivity reactions via Ab-mediated cellular dysfunction?

A

Ab inhibits binding of NT or hormone to receptor

Disrupts endocrine and neural signaling

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43
Q

What Type II Hypersensitivity reactions are caused by Ab-mediated cellular dysfunction? What are their targets?

A

Myasthenia Gravis - Target ACh receptor, inhibit ACh binding

Graves disease - Target and stimulate TSH receptor

Insulin-resistant diabetes - target Insulin Receptor, inhibit insulin binding

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44
Q

What is the basic mechanism of Type III Hypersensitivity reactions?

A
  1. Ab combines w/ excess soluble Ag, forming large complexes
  2. Circulating immune complexes become lodged in BM of epithelia (kidney, lungs, joints, skin)
  3. Fragments of complement cause histamine and other mediator release
  4. Neutrophils migrate to site of immune complex deposition => release enzymes => tissue and organ damage
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45
Q

What type of hypersensitivity are the arthus reaction and serum sickness? What are the target Ag?

A

Type III Hypersensitivity

Serum sickness (acute) - Non-human ptn Ag such as diphtheria antitoxin

Serum sickness (chronic) - self Ag

Arthus rxn (local) - rare local effect of vaccination

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46
Q

If immunofluorescence is performed on biopsies, how would you differentiate between hypersensitivity reactions?

A

Pattern of Ab deposition:

Type II: Smooth, linear

Type III: Grainy, granular

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47
Q

Where do post-streptococcal cross-reactive Ab act in a Type II reaction? In a Type III reaction?

A

Type II - act directly on myocardium

Type III - Form immune complexes that deposit in the glomeruli

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48
Q

What is the hallmark of a Type IV Hypersensitivity reaction?

A

Immune granulomas: caused by a variety of agents that induce persistent T cell-mediated immune responses

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49
Q

Rheumatoid arthritis, multiple sclerosis, IBS, Type I DM, Psoriasis, and contact sensitivity are all examples of what type of hypersensitivity?

A

Type IV Hypersensitivity

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50
Q

In general, what cytokines mediate Type IV Hypersensitivity reactions?

A

Th1 and TH17

(except Type I DM - destruction by CTLs, Psoriasis - mainly TH17)

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51
Q

If T Cell receptors react with Self-Ag in the thymus, what happens to them?

A

Apoptosis

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52
Q

If T Cell receptors react with Self-Ag in the bone marrow, what happens to them?

A

They either edit their receptor and continue or undergo apoptosis

(Bend the knee or die)

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53
Q

What T cell receptors promote anergy?

A

CTLA and PD-1

(Help T cells downregulate when self-Ag present, utilized by viruses and tumors => blocking CTLA and PD-1 binding can intensify immune response against CA cells)

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54
Q

What are Treg cells induced by? What CD do they recognize? What genes do they express?

A

Induced by TGF-B

Positive for CD4

Express CD25 and FoxP3

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55
Q

What is inhibited when Treg cells are suppressed?

A

Release of IL-10 and TGF-β

Expression of CTLA-4

(supresses maternal response to allow foreign fetus to grow)

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56
Q

What is the purpose of AIRE gene? Where does it exert its normal function?

A

Stimulate expression of proteins that are not normally expressed in the thymus, critical for central tolerance (deletion of immature T cells specific for these Ag)

Exerts function in the thymus

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57
Q

What normal process would fail in the absence of normal AIRE? Resulting in what disease process?

A

Central tolerance

Failure/Mutations in AIRE gene => autoimmune polyendocrinopathy

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58
Q

What does IPEX stand for?

A

I - Immune dysregulation

P - Polyendocrinopathy

E - Enteropathy

X - X-linked

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59
Q

What mutation is involved in IPEX disease?

A

FOXP3

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60
Q

What normal process fails in IPEX disease?

A

Development and maintenance of functional CD4 helper T cells

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61
Q

What are the 3 criteria for defining pathological autoimmunity?

A
  1. Immune reaction directed against a self-Ag
  2. Immune rxn primarily responsible for a pathologic condition
  3. No other pathophysiology possible
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62
Q

Upon imaging, a pt with back pain shows inflammation in the joints and fusion of the vertebrae. What disease process is this?

A

Ankylosing spondylitis

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63
Q

What gene is ankylosing spondylitis strongly associated with?

A

Class I HLA allele B27

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64
Q

Ankylosing spondylitis generally affects what population?

A

Young males with family hx of inflammatory joint conditions

65
Q

Polymorphisms in what gene will ultimately lead to Crohn’s disease? What process is rendered defective?

A

NOD-2 => defective NOD-like receptor => ineffective innate immune response

Paneth cells become ineffective at microbe killing => accumulation of bacteria => exaggerated immune response => Tissue damage and ulcers

66
Q

What is the disease response process in Oral Lichen Planus?

A

Epitope spreading:

Initial T cell response causes lesions in keratin in the oral and conjunctival mucosa => Basement membrane disruption reveals new Ag => secondary response causes blistering in secondary pemphigoid

67
Q

What is the first test you would get for a pt with suspected lupus, Sjogren syndrome, or systemic sclerosis?

A

Anti-nuclear antibody (ANA)

(sensitive test for multiple autoimmune diseases, can prompt more specific testing to confirm Dx)

68
Q

An ANA immunofluorescent stain comes back with a homogeneous appearance on the cells. what disease process is most likely?

A

Systemic Lupus Erythematosus (SLE)

69
Q

What is the gene associated with SLE?

A

HLA-DQ

70
Q

What populations are affected by SLE?

A

Female

Family hx of SLE

People who are chronically exposed to UV light

71
Q

Pt presents with malar rash, fever, fatigue, and edema in the legs. Upon testing, pt has hemolytic anemia and a positive ANA. What disease does she most likely have?

A

SLE

72
Q

What are the most common signs and symptoms of lupus? (a lot)

A

Malar rash

Hair loss

Swollen LNs

Chest pain

Muscle aches

Inflammation and damage to kidneys, lungs, heart, BVs, CNS

Raynaud’s in fingers and toes

Arthritis/joint pain

Fever, fatigue

photosensitive

Anemia

73
Q

What stage do people normally present to their physician with Lupus Nephritis? What is the terminal stage of lupus?

A

Typically present with diffuse lupus nephritis

Sclerosing nephritis (fibrotic) is terminal

74
Q

What is the appearance of the glomeruli in diffuse Lupus Nephritis (IV)?

A

Increased proliferation of endothelial, mesangial, and epithelial cells

Immune deposits in the subendothelium (grainy/granular IF appearance as in a Type III Hypersensitivity due to IgG ab-containing complexes)

75
Q

What occurs in Libman-Sacks Endocarditis? What disease is this a complication of?

A

“Verrucous” (warty) valve deposits composed of fibrin

Not infective

Can embolize (rarely)

Complication of SLE

76
Q

What type of cell seen in SLE is seen as a neutrophil/macrophage ingesting the nucleus of a damaged cell?

A

L-E cell

May be seen in blood/body fluids

77
Q

What are the main diagnostic features of Discoid Lupus Erythematosus?

A

Discoid rash

Positive ANA

Positive immunofluorescence

Negative Anti-DS DNA

78
Q

What are the main diagnostic features of Drug-Induced Lupus Erythematosus?

A

Arthralgias (joint aches/fever)

Positive ANA

Discoid rash

Hematologic disease

Positive immunofluorescence

79
Q

What type of lupus is associated with a positive Anti-Histone Ab?

A

Drug-Induced Lupus

80
Q

What drugs cause Drug-Induced Lupus? What genes do they interfere with?

A

Hydralazine (anti-arrhythmic) - HLA-DR4

Procainamide (BP med) - HLA-DR6

81
Q

What glandular tissue is targeted in Sjogren syndrome?

A

Lacrimal and salivary glands

82
Q

What is the clinical presentation for Sjogren syndrome?

A

Dry, irritated “gritty” eyes

Dry mouth (xerostomia) => yeast infection and root caries

83
Q

What Ab are tested for in Sjogren syndrome?

A

Anti-Ro/SS-A

Anti-La/SS-B

84
Q

What are the main complications of Sjogren syndrome?

A

Formation of extraglandular tissue - pulmonary fibrosis

Lymphoid proliferation becoming clona => lymphoma

85
Q

How does systemic sclerosis present on a histological slide?

A

Dense collagenous deposition

Vascular hyalinization

86
Q

Why does systemic sclerosis cause raynaud’s phenomena?

A

Sclerosis of the vasculature in the fingers cuts off blood supply => necrosis

87
Q

How does the ANA immunofluorescent pattern present in systemic sclerosis?

A

Speckled

88
Q

How does the ANA immunofluorescent pattern present in CREST syndrome?

A

Centromeric

(Ab attack the centromeres of the cells)

89
Q

What does CREST syndrome encompass?

A

C - Calcinosis/calcium deposits in the skin

R - Raynaud’s phenomenon (spasm of BVs in response to cold or stress)

E - Esophageal dysfunction => acid reflux

S - Sclerodactyly: thickening and tightening of skin on fingers and hands

T - Telangiectasias: dilation of capillaries causing red marks on surface of skin

(better prognosis than diffuse sclerosis)

90
Q

What are the hallmarks for Mixed Connective Tissue Disease?

A

High titer for Anti-ribonucleoprotein (RNP)

Commonly presents w/ raynaud’s phenomenon

91
Q

What are the IgG4-related diseases?

A

Autoimmune pancreatitis

Riedel thyroiditis

Mikulicz’s syndrome (autoimmunity of salivary glands)

Idiopathic retroperitoneal fibrosis (in ureters => kidney failure but it is all up and down the retroperitoneum)

Inflammatory pseudotumors

Inflammatory aortitis

92
Q

What is the pathophysiology of IgG4 related disease?

A

IgG4-producing plasma cells, T lymphocytes causing irreversible tissue damage and fibrosis

93
Q

What is the main process behind transplant rejection?

A

Allorecognition (body recognizes transplant as non-self)

94
Q

How does the direct pathway of allorecognition affect tissue graft?

A

Dendritic cells in the donor organs become APCs for initiating anti-graft response

95
Q

How does the indirect pathway of allorecognition affect tissue grafts?

A

Recipients APC recognize MHC Ag on graft donors and present this Ag to its own CD4 cells => activate macrophages or B cells => plasma cells to attack BVs

96
Q

When does hyperacute graft rejection occur?

A

Minutes to hours

Recipient has pre-formed Ab to the donor tissue => inflammation followed by thrombotic microvasculopathy

97
Q

What is the breakdown product in Acute Ab-mediated tissue rejection?

A

Complement C4d breakdown product

98
Q

When does acute cellular tissue rejection occur? What are the mediators of this process?

A

Days, months, years of transplant

T-Cell mediated

99
Q

What drugs are used to prevent Ab-mediated rejection of transplatns?

A

Immune globulin

Rituximab (anti CD20 recombinant Ab)

100
Q

What is the drug used to treat T-lymphocyte mediated cellular rejection?

A

Tacrolimus

101
Q

What infections are transplant pts at higher risk for?

A

Viral: Polyomavirus, cytomegalovirus

Fungal

Bacterial

102
Q

What types of tumors are transplant pts at increased risk for?

A

Viral-induced tumors: lymphomas, Kaposi sarcoma

Squamous cell carcinoma (skin)

103
Q

Graft Vs Host Disease is mediated by what cells?

A

T Lymphocytes

104
Q

What are common infectious consequences of B cell deficiencies?

A

Pyogenic bacterial infections

Enteric bacterial and viral infections

105
Q

What are common infectious consequences of T cell deficiencies?

A

Viral and other intracellular microbial infections (pneumocystitis jiroveci)

Some cancers (EBV-associated lymphomas, skin cancers)

106
Q

What is defective in Chediak Higashi syndrome?

A

Failure of phagolysosomal fusion => Increased bacterial infections

(AR primary leukocyte disorder, fatal w/o stem cell transplant)

107
Q

Defects in melanocytes leading to albinism in pts and a giant granule appearance on histological slides can be indicative of what disorder?

A

Chediak Higashi syndrome

108
Q

What mechanism is defective in chronic granulomatous disease?

A

Failure of superoxide production w/in phagocytes => accumulation of macrophages => wall off infection

(primary leukocyte disorder)

109
Q

Failure of what components cause MAC deficiency? What infections does this commonly lead to?

A

Deficiency in C5, 6, 7, 8, 9

Neisseria infection => meningitis

110
Q

Hereditary angioedema is caused by deficiency in what?

A

C1 inhibitor

(AD inheritance)

111
Q

What enzyme is most commonly defective in AR-inherited SCID?

A

Adenosine deaminase deficiency

112
Q

What is defective in X-Linked SCID?

A

Mutations in IL receptors => reduced # of T cells

113
Q

What is deficient/failed in DiGeorge syndrome?

A

Failure of pharyngeal pouches 3 and 4 => failure of development of thymus, parathyroids, heart, and great vessels => Deficient in mature T lymphocytes

114
Q

What chromosome has been deleted to produce DiGeorge syndrome?

A

22q11

115
Q

What disease process can result in Tetralogy of Fallot, truncus arteriosus, and tetany?

A

DiGeorge Syndrome

116
Q

What enzyme is deficient in X-linked agammaglobinemia?

A

Bruton tyrosine kinase (gene defective on X chromosome) => Inability of B cells to mature

117
Q

What infections are pts with agammaglobulinemia susceptive to?

A

Encapsulated bacteria: Strep pneumoniae, haemophilus influenzae Type B, streptococcus pyogenes, pseudomonas aeruginosa, staph aureus

Viruses

Protozoa: giardia lamblia

118
Q

What failed in Hyper IgM syndrome?

A

CD40/CD40L mutations => interfere w/ T cells helping B cells to class switch

119
Q

What is the most common significant primary immunodeficiency?

A

Common Variable Immunodeficiency (CVID) - disease of exclusion

120
Q

How do pts with CVID present?

A

Older pts (after months after birth)

Recurrent encapsulated bacterial infections (sinus and pulmonary infections)

Granulomas

Chronic diarrhea (giardia lamblia)

Autoimmune disease: anemia, thrombocytopenia

121
Q

Pt presents with an anaphylactic reaction after a red cell transfusion. He states he has a history of frequent infections and allergies. What does this patient most likely have?

A

IgA deficiency

122
Q

What gene is mutated in Wiskott Aldrich syndrome? What sx are characteristic?

A

WASP gene mutation

Triad of sx: thrombocytopenia, eczema, recurrent infections

123
Q

What is deficient in Ataxia Telangiectasia?

A

ATM gene mutation => defective gene repair => immune deficiency (def IgA and IgG) => resp infections, autoimmune dz, CA

124
Q

AIDS is the manifestation of when HIV affects immune function to the point of what 3 things?

A
  1. Opportunistic infections
  2. Secondary neoplasms
  3. Neurologic manifestations
125
Q

Who are the at-risk populations for HIV?

A

Homosexual/bisexual men

IV drug users

Hemophiliacs

Other recipients of blood/blood components

Heterosexual contacts of above groups

Newborns in areas with high female HIV prevalence

126
Q

What is the capsid protein tested for in HIV?

A

P24 (the 2nd detectable substance for HIV)

127
Q

What are the glycoproteins important for HIV attachment to cells? What can they be used as?

A

GP120 and GP41

Good drug/vaccine targets

128
Q

What are the major regions of the HIV-1 genome to know? What do they encode?

A

Long terminal repeat (LTR): initiates txn, binds txn factors

GAG: encodes ptn inside virus

ENV: encodes for surface glycoptns

Pol: encodes viral enzymes (polymerase)

129
Q

Life cycle of HIV

A
  1. Virus entry using CD4 molecule as a receptor (co-receptors may include CCR5 and CXCR4)
  2. GP120 binding => conformational change
  3. GP120, CD4 bind CCR-5
  4. GP41 membrane penetration
  5. Membrane fusion => entry of viral genome into cytoplasm
  6. Reverse transcriptase-mediated synthesis of proviral DNA
  7. Integration of provirus into host cell genome via integrase =>
  8. Host cell activation => LTR initiates TXN
  9. Synthesis of HIV ptns
  10. Budding and release of mature virion
130
Q

In the case of HIV, why is antigenic stimulation counter-intuitive to the host immune response?

A

Ag stimulation is supposed to release NFkB => upregulate the T cell response but actually initiates viral txn through the LTR

131
Q

What is the direct cytopathic effect in HIV?

A

Viral replication in the cell is responsible for host cell/CD4 death

132
Q

What is pyroptosis?

A

Inflammasome-mediated programmed death pathway responsible for cell death in non-replicating viral infection

133
Q

How does HIV tropism affect macrophages?

A

Macrophages become HIV reservoir and become resistant to cytopathic effects

134
Q

How does HIV tropism affect DCs?

A

Mucosa - used for transport to LNs

LNs: additional reservoirs for HIV

135
Q

How does HIV tropism affect microglia?

A

Infection in monocytes to microglial cells in brain allows HIV access to CNS

Neuronal damage from viral product

136
Q

What happens to B lymphocytes in HIV infection?

A

Proliferative response (might be due to secondary infection)

May become clonal (lymphoma)

Non-specific hypergammaglobinemia

Impaired humoral immunity

137
Q

How long is the acute viremic period of HIV?

A

2-3 weeks

138
Q

When does the the acute retroviral syndrome spike of HIV occur? What occurs during this phase?

A

3-7 weeks after infection

Seroconversion - developing anti-HIV Ab

139
Q

What is the sequence of detectable substances in HIV testing?

A

1st detected: Viral RNA via Nucleic Acid Test (NAT)

2nd detected: Ptn Ag p24

3rd: HIV Ab

140
Q

What is the window period for HIV testing?

A

The period of time (7-14 days) that it takes to test donated blood and blood products for HIV infection

141
Q

What is the clinical signifiance of the viral set point?

A

Signifies the end of the initial viremic spike

May predict the CD4 cell loss and latency period

142
Q

What diseases manifest in AIDS?

A

Opportunistic infections (pneumocystis PNA, TB, toxoplasma, candida mucositis, cryptosporidium diarrhea)

Neoplasia (kaposi sarcoma, lymphoma)

143
Q

What opportunistic fungal infection is the AIDS defining illness?

A

Pneumocystis jiroveci (carinii)

144
Q

What virus (along with HIV) is associated with the development of Kaposi sarcoma?

A

HHV 8 (EBV reactivated)

145
Q

How do AIDS lymphomas develop?

A

HIV allows opportunistic viral infection (EBV reactivation)

HIV decreases cell immunity which usually checks B cell proliferation

146
Q

What CA is AIDS-defining?

A

Cervical CA (infection by HPV)

147
Q

What are the systemic amyloidoses and their associated fibril ptn?

A

Immunocyte dyscrasias w/ amyloidosis (primary) - AL

Reactive systemic amyloidosis (secondary) - AA

Hemodialysis-associated amyloidosis

148
Q

What are the hereditary amyloidoses and their associated fibril ptn?

A

Famiial Mediterranean fever - AA

Familial amyloidotic neuropathies - ATTR

Systemic senile amyloidosis - ATTR

149
Q

What are the localized amyloidoses?

A

Senile cerebral - Alzheimer’s

Endocrine - Type 2 DM

Medullary carcinoma of thyroid

Islets of langerhans

Isolated atrial amyloidosis

150
Q

What is the initial presentation of amyloid in the kidney?

A

Edema due to disruption of glomeruli and resulting proteinuria

151
Q

What is the initial presentation of amyloid in the heart?

A

Dysrhythmias due to disruption of myocardium

152
Q

What stain is used to demonstrate presence of amyloid? What color will it appear as?

A

Congo red stain

Appears apple green

153
Q

What Ab are tested for in lupus?

A

Anti DS DNA

Anti Smith

154
Q

What Ab are tested for in systemic sclerosis?

A

Anti-DNA Topoisomerase (Scl-70)

155
Q

Failure of immune cell development in what structure leads to X-linked agammaglobulinemia?

A

Bone marrow

156
Q

Failure of immune cell development in what structure leads to ADA deficiency, X-linked SCID, DiGeorge, MHC class II deficiency?

A

Thymus

157
Q

Failure of immune cell development in what structure leads to CVID, Hyper-IgM syndrome, IgA def?

A

Blood circulation

158
Q

What are the most common locations of amyloid to manifest?

A

Brain

Kidney

Liver

Heart

Fat pads

MOD Exam 2 (9 decks)

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