immunology Flashcards

1
Q

Interleukin-1 (IL-1)

A

Function: Causes fever, acute inflammation. Activates endothelium to express adhesion molecules. Induces chemokine secretion to recruit WBCs. Also called osteoclast-activating factor.
Mnemonic: Hot T-bone stEAK (IL-1: fever [hot])

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2
Q

What is the function of Interleukin-1 (IL-1)?

A

Causes fever, acute inflammation. Activates endothelium to express adhesion molecules. Induces chemokine secretion to recruit WBCs. Also called osteoclast-activating factor.

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3
Q

What is the mnemonic for Interleukin-1 (IL-1)?

A

Hot T-bone stEAK (IL-1: fever [hot])

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4
Q

What is the function of Interleukin-2 (IL-2)?

A

Stimulates growth of helper, cytotoxic, and regulatory T cells, and NK cells.

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5
Q

What is the mnemonic for Interleukin-2 (IL-2)?

A

T-bone stEAK (IL-2: stimulates T cells)

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6
Q

What is the function of Interleukin-3 (IL-3)?

A

Supports growth and differentiation of bone marrow stem cells. Functions like GM-CSF.

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7
Q

What is the mnemonic for Interleukin-3 (IL-3)?

A

T-bone stEAK (IL-3: stimulates bone marrow)

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8
Q

What is the function of Interleukin-4 (IL-4)?

A

Induces differentiation of T cells into Th2 cells. Promotes growth of B cells. Enhances class switching to IgE and IgG.

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9
Q

What is the mnemonic for Interleukin-4 (IL-4)?

A

Ain’t too proud 2 BEG 4 help (IL-4)

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10
Q

What is the function of Interleukin-5 (IL-5)?

A

Promotes growth and differentiation of B cells. Enhances class switching to IgA. Stimulates growth and differentiation of eosinophils.

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11
Q

What is the mnemonic for Interleukin-5 (IL-5)?

A

I have 5 BAEs

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12
Q

What is the function of Interleukin-6 (IL-6)?

A

Causes fever and stimulates production of acute-phase proteins.

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13
Q

What is the mnemonic for Interleukin-6 (IL-6)?

A

T-bone stEAK (IL-6: stimulates aKute-phase protein production)

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14
Q

What is the function of Tumor Necrosis Factor-α (TNF-α)?

A

Activates endothelium. Causes WBC recruitment, vascular leak. Causes cachexia in malignancy. Maintains granulomas in TB. Can mediate fever and sepsis.

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15
Q

What is the function of Interleukin-8 (IL-8)?

A

Major chemotactic factor for neutrophils.

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16
Q

What is the mnemonic for Interleukin-8 (IL-8)?

A

Clean up on aisle 8

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17
Q

What is the function of Interleukin-12 (IL-12)?

A

Induces differentiation of T cells into Th1 cells. Activates NK cells. Facilitates granuloma formation in TB.

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18
Q

What is the function of Interferon-γ (IFN-γ)?

A

Secreted by NK cells and T cells in response to antigen or IL-12 from macrophages. Stimulates macrophages to kill phagocytosed pathogens. Inhibits differentiation of Th2 cells. Induces IgG isotype switching in B cells. Increases MHC expression and antigen presentation by all cells. Activates macrophages to induce granuloma formation.

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19
Q

What is the function of Interleukin-10 (IL-10)?

A

Attenuates inflammatory response. Decreases expression of MHC class II and Th1 cytokines. Inhibits activated macrophages and dendritic cells. Also secreted by regulatory T cells.

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20
Q

What is the function of Interleukin-13 (IL-13)?

A

Promotes IgE production by B cells. Induces alternative macrophage activation.

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21
Q

What is the mnemonic for Interleukin-13 (IL-13)?

A

Interleukin thirtEEn promotes IgE

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22
Q

What is the defect in X-linked (Bruton) Agammaglobulinemia?

A

Defect in BTK, a tyrosine kinase gene → no B-cell maturation; X-linked recessive (↑ in Boys)

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23
Q

What is the presentation of X-linked (Bruton) Agammaglobulinemia?

A

Recurrent bacterial and enteroviral infections after 6 months (↓ maternal IgG)

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24
Q

What are the findings in X-linked (Bruton) Agammaglobulinemia?

A

Absent B cells in peripheral blood, ↓ Ig of all classes, absent/scanty lymph nodes and tonsils (1° follicles and germinal centers absent)

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25
What is the defect in Selective IgA Deficiency?
Unknown
26
What is the presentation of Selective IgA Deficiency?
Majority Asymptomatic; can see airway and GI infections, autoimmune disease, atopy, anaphylaxis to IgA in blood products
27
What are the findings in Selective IgA Deficiency?"
↓ IgA with normal IgG, IgM levels; ↑ susceptibility to giardiasis
28
What is the defect in Common Variable Immunodeficiency?
Defect in B-cell differentiation. Cause unknown in most cases
29
What is the presentation of Common Variable Immunodeficiency?
May present in childhood but usually diagnosed after puberty. ↑ risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections
30
What are the findings in Common Variable Immunodeficiency?
↓ plasma cells, ↓ immunoglobulins
31
What is the defect in Thymic Aplasia (DiGeorge Syndrome)?
22q11 microdeletion; failure to develop 3rd and 4th pharyngeal pouches → absent thymus and parathyroids
32
What is the presentation of Thymic Aplasia (DiGeorge Syndrome)?
CATCH-22: Cardiac defects, Abnormal facies, Thymic hypoplasia, Cleft palate, Hypocalcemia
33
What are the findings in Thymic Aplasia (DiGeorge Syndrome)?
↓ T cells, ↓ PTH, ↓ Ca2+, thymic shadow absent on CXR
34
What is the defect in IL-12 Receptor Deficiency?
↓ Th1 response; autosomal recessive
35
What is the presentation of IL-12 Receptor Deficiency?
Disseminated mycobacterial and fungal infections; may present after administration of BCG vaccine
36
What are the findings in IL-12 Receptor Deficiency?"
↓ IFN-γ
37
What is the defect in Hyper-IgE Syndrome (Job Syndrome)?
Deficiency of Th17 cells due to STAT3 mutation → impaired recruitment of neutrophils to sites of infection
38
What is the presentation of Hyper-IgE Syndrome (Job Syndrome)?
Cold staphylococcal abscesses, retained baby teeth, coarse facies, eczema, ↑ IgE, bone fractures from minor trauma
39
What are the findings in Hyper-IgE Syndrome (Job Syndrome)?"
↑ IgE, ↑ eosinophils
40
What is the defect in Chronic Mucocutaneous Candidiasis?
T-cell dysfunction; impaired cell-mediated immunity against Candida sp.
41
What is the presentation of Chronic Mucocutaneous Candidiasis?
Persistent noninvasive Candida albicans infections of skin and mucous membranes
42
What are the findings in Chronic Mucocutaneous Candidiasis?"
Absent in vitro T-cell proliferation in response to Candida antigens, absent cutaneous reaction to Candida antigens
43
What is the defect in Severe Combined Immunodeficiency (SCID)?
Several types including defective IL-2R gamma chain (most common, X-linked recessive); adenosine deaminase deficiency (autosomal recessive); RAG mutation → VDJ recombination defect
44
What is the presentation of Severe Combined Immunodeficiency (SCID)?
Failure to thrive, chronic diarrhea, thrush, recurrent infections
45
What are the findings in Severe Combined Immunodeficiency (SCID)?"
↓ T-cell receptor excision circles (TRECs), absence of thymic shadow, germinal centers, and T cells
46
What is the defect in Ataxia-Telangiectasia?
Defects in ATM gene → failure to detect DNA damage → failure to halt cell cycle progression → mutations accumulate; autosomal recessive
47
What is the presentation of Ataxia-Telangiectasia?
Triad: cerebellar defects (Ataxia), spider Angiomas (telangiectasia), IgA deficiency
48
What are the findings in Ataxia-Telangiectasia?"
↑ AFP, ↓ IgA, IgG, and IgE, lymphopenia, cerebellar atrophy
49
What is the defect in Hyper-IgM Syndrome?
Most commonly due to defective CD40L on Th cells → class switching defect; X-linked recessive
50
What is the presentation of Hyper-IgM Syndrome?
Severe pyogenic infections early in life; opportunistic infection with Pneumocystis, Cryptosporidium, CMV
51
What are the findings in Hyper-IgM Syndrome?"
Normal or ↑ IgM, ↓ IgG, IgA, IgE, failure to make germinal centers
52
What is the defect in Wiskott-Aldrich Syndrome?
Mutation in WAS gene; leukocytes and platelets unable to reorganize actin cytoskeleton → defective antigen presentation; X-linked recessive
53
What is the presentation of Wiskott-Aldrich Syndrome?
WATER: Wiskott-Aldrich: Thrombocytopenia, Eczema, Recurrent infections; ↑ risk of autoimmune disease and malignancy
54
What are the findings in Wiskott-Aldrich Syndrome?"
↓ to normal IgG, IgM; ↑ IgE, IgA; fewer and smaller platelets
55
What is the defect in Leukocyte Adhesion Deficiency (Type 1)?
Autosomal recessive defect in LFA-1 integrin (CD18) protein on phagocytes leads to impaired migration and chemotaxis by C5a, IL-8, and leukotriene B4
56
What is the presentation of Leukocyte Adhesion Deficiency (Type 1)?
Late separation (>30 days) of umbilical cord, absent pus, dysfunctional neutrophils → recurrent skin and mucosal bacterial infections
57
What are the findings in Leukocyte Adhesion Deficiency (Type 1)?"
↑ neutrophils in blood, absence of neutrophils at infection sites
58
What is the defect in Chédiak-Higashi Syndrome?
Defect in lysosomal trafficking regulator gene (LYST); microtubule dysfunction in phagosome-lysosome fusion; autosomal recessive
59
What is the presentation of Chédiak-Higashi Syndrome?
PLAIN: Progressive neurodegeneration, Lymphohistiocytosis, Albinism (partial), recurrent pyogenic Infections, peripheral Neuropathy
60
What are the findings in Chédiak-Higashi Syndrome?"
Giant granules in granulocytes and platelets, pancytopenia, mild coagulation defects
61
What is the defect in Chronic Granulomatous Disease?
Defect of NADPH oxidase → ↓ reactive oxygen species (e.g., superoxide) and ↓ respiratory burst in neutrophils; X-linked form most common
62
What is the presentation of Chronic Granulomatous Disease?
↑ susceptibility to catalase-positive organisms, recurrent infections, and granulomas
63
What are the findings in Chronic Granulomatous Disease?"
Abnormal dihydrorhodamine (flow cytometry) test (↓ green fluorescence), nitroblue tetrazolium dye reduction test fails to turn blue