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Step 1 Flash Cards > Immunology > Flashcards

Immunology Flashcards

1
Q

HLA A3

A

hemochromatosis

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2
Q

HLA B27

A

PAIR

- psoriatic arthritis, ank spon, IBD, reactive arthritis

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3
Q

HLA DQ2/DQ8

A

celiac disease

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4
Q

HLA DR2

A

MS, hay fever, SLE, goodpasture, syndrome

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5
Q

HLA DR3

A

DM 1, SLE, Graves Disease

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6
Q

HLA DR4

A

RA, DM 1

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7
Q

HLA DR5

A

pernicious anemia, Hashimoto thyroiditis

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8
Q

IL-1

A

secreted by macrophages. endogenous pyrogen, also called osteoclast activating factor. causes fever, acute inflammation. activates endothelium to express adhesion molecules, induces cytokine secretion to recruit leukocytes

  • “Hot T-bone stEAK”
  • stimulates hot (fever), T cells, bone marrow, IgE, IgA, aKute phase reactants production
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9
Q

IL-2

A

secreted by all T cells, stimulates growth of helper, cytotoxic and regulatory T cels
- can be used for tumor regression in RCC and metastatic melanoma

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10
Q

IL-3

A

secreted by all T cells

- support the growth and differentiation of BM stem cells. functions like GM-CSF

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11
Q

IL-4

A

secreted by TH2 cells

  • induces differentiation into TH2 cells, promotes growth of B cells
  • enhances class switching to IgG and IgE
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12
Q

IL-5

A

secreted by TH2 cells

  • promotes differentiation of B cells, enhances class switching to IgA
  • stimulates the growth and differentiation of eosinophils
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13
Q

IL-6

A

secreted by macrophages and TH2 cells

  • an endogenous pyrogen
  • fever and stimulates production of acute phase proteins
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14
Q

IL-8

A

secreted my macrophages

- major chemotactic factor for neutrophils

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15
Q

IL-10

A

secreted by TH2 cells and Treg

  • modulates inflammatory response, inhibits actions of activated T cells and Th1
  • enhances IL4 and 5 secretion from TH2
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16
Q

IL-12

A

secreted by macrophages and B cells

  • induces differentiation of T cells into Th1 cells
  • activates NK cells
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17
Q

TNF-alpha

A

secreted by macrophages

  • mediates septic shock, activates endothelium, causes leukocyte recruitment and vascular leak
  • mediates cachexia
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18
Q

IFN gamma

A

secreted by Th1 cells

  • has antiviral and antitumor properties
  • activates NK cells
  • increases MHC expression and antigen presentation in all cells
  • used therapeutically in CGD
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19
Q

INF alpha and beta

A
  • interferes with viral replication by halting protein synthesis in virally infected cells
  • essentially results in apoptosis
  • alpha used therapeutically in Hep B/C, Kaposi sarcoma, hairy cell leukemia, condyloma acuminatum, RCC and malignant melanoma
  • beta used therapeutically in MS
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20
Q

T cell surface receptors

A
  • TCR, CD3, CD28 (binds to B7 in APC)
  • Helper T cells have CD4 and CD40 ligand
  • Cytotoxic T cells have CD8
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21
Q

B cell surface receptors

A
  • CD19, CD20, CD21 (EBV entry), CD40

- MHC II, B7

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22
Q

macrophage cell surface receptors

A
  • CD 14, CD 40
  • MHC II, B7
  • Fc and C3b receptors
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23
Q

NK cell surface receptors

A
  • CD 16 (binds Fc of IgG)

- CD 56 (unique marker for NK)

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24
Q

Type I HS reaction

A
  • anaphylactic and atopic - free antigen cross links with IgE (aggregation of IgE-Fc receptors triggers degranulation) on mast cells/basophils triggering immediate release of histamine and tryptase (sensitive for mast cell activation)
  • delayed response due to leukotrienes
  • test: skin test for IgE
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25
Q

Type II HS reaction

A
  • cytotoxic “cy-2-toxic” - IgG and IgM bind fixed antigen on enemy cell, leading to cellular destruction
  • 3 mech: opsonization (phagocytosis or complement), complement mediated lysis (transfusion reaction), Ab dependent cell mediated tox with NK or macrophage
  • test: direct and indirect Coombs
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26
Q

Coombs test

A
  • direct tests for antibodies that have adhered to antigen (test Rh + baby with Rh- mom)
  • indirect tests for antibodies that can adhere to other RBCs (test Rh - mom for Abs)
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27
Q

Type III HS reaction

A
  • immune complex - IgG complexes activate complement, which attracts neutrophils that release lysosomal enzymes
  • ex: serum sickness, arthus reaction
  • III - 3 things stuck together - antigen-antibody-complement
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28
Q

serum sickness

A

immune complex idsease in which Abs to the foreigh proteins are produced (takes 5 days)

  • immune complexes form and are deposited in membranes, where they fix complement –> tisue damage
  • assoc with monoclonal Ab drugs
  • fever, urticaria, arthralgias, proteinuria, lymphadenopathy 5-10 days after antigen exposure
  • small vessel vasculitis with fibrinoid necrosis and intense neutrophil infiltration
  • hypocomplementemia
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29
Q

arthus reaction

A
  • local subacute type III HS reaction
  • interdermal injection of antigen induces antibodies, which form complexes in the skin
  • edema, necrosis and activation of complement
  • test: IF staining
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30
Q

Type IV HS reaction

A
  • delated (T cell mediated)
  • sensitized T cells encounter antigen and then release lymphokines (leads to macrophage activation, no Ab involved)
  • 4 T’s - T lymphocytes, Transplant rejections, TB skin tests, Touching (contact derm)
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31
Q

What type of reactions characterize each HS reaction?

A

ACID

  • I - Anaphylactic and Atopic
  • II - Cytotoxic
  • III - Immune complexes
  • IV - Delayed (cell mediated)
32
Q

Type I HS disorders

A
  • anaphylaxis

- allergic and atopic disorders (rhinitis, hay fever, eczema, hives, asthma)

33
Q

Type II HS disorders

A
  • AI hemolytic anemia
  • pernicious anemia
  • ITP
  • erythroblastosis fetalis
  • acute hemolytic transfusion reactions
  • Rheumatic fever
  • Goodpasture syndrome
  • Myasthenia gravis
  • Bullous Pemphigoid
  • Pemphigus Vulgaris
34
Q

Type III HS disorders

A
  • SLE, polyarteritis nodosa, PSGN, serum sickness, arthus reaction (swelling and inflammation following tetanus vaccine)
35
Q

Type IV HS disorder

A
  • MS, GBS, Graft v. Host disease, PPD, contact derm
36
Q

allergic blood transfusion reaction

A
  • Type I HS reaction against plasma proteins in transfused blood
  • urticaria, pruritis, wheezing, fever
  • treat with antihistamines
37
Q

anaphylactic blood transfusion reaction

A
  • severe allergic reaction
  • IgA deficient pts need blood without IgA or this will happen
  • dyspnea, bronchospasm, hypotension, respiratory arrest, shock
38
Q

febrile nonhemolytic transfusion reaction

A
  • Type II HS reaction
  • host Abs against donor HLA antigens and leukocytes
  • fever, headaches, chills, flushing
39
Q

acute hemolytic transfusion reaction

A
  • Type II HS reaction
  • intravascular hemolysis due to ABO incompatibility or extravascular hemolysis due to host Ab reaction against foreign antigen on donor RBCs
  • complement mediated
  • fever, hypotension, tachypnea, tachycardia, flank pain, hemoglobinemia and jaundice
40
Q

Bruton agammaglobulinemia

A
  • X linked recessive defect in BTK - a tyrosine kinase gene - no B cell maturation
  • presents with recurrent bacterial, enteroviral and giardia infections after 6 months
  • normal CD19+ B cell count, dec pro-B, dec Ig of all classes
  • absent/scanty lymph nodes and tonsils with no germinal centers
41
Q

selective IgA deficiency

A
  • most common primary immunodeficiency
  • can see airway and GI (mucosal) infections, autoimmune disease (assoc with celiac), atopy, anaphylaxis to IgA containing products
  • IgA < 7 mg/dL with normal IgG and IgM levels
42
Q

common variable immunodeficiency

A
  • defect in B cell differentiation due to B cell or helper T cell defect (cant make immunoglobulins)
  • can be acquired in 20s-30s, inc risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections
  • bacterial, enterovirus and giardia infections
  • decreased plasma cells, decreased immunoglobulins
43
Q

C1 esterase inhibitor deficiency

A
  • causes hereditary angioedema

- NO ACE inhibitors

44
Q

C3 deficiency

A

increases risk of severe, pyogenic sinus and resp tract infections, inc susceptibility to type III HS reactions

45
Q

C5-C9 deficiencies

A
  • increased susceptibility to Neisseria infections, no ability to make MAC
46
Q

DAC (GP1 anchored enzyme) deficiency

A
  • causes complement mediated lysis of RBCs and PNH
47
Q

Thymic aplasia (DiGeorge)

A
  • 22q11 deletion, failure of 3rd and 4th pharyngeal pouches (no thymus or parathyroids)
  • presents with hypocalcemia, recurrent viral/fungal infections, conotruncal abnormalities (ToF, truncus arteriosus)
  • dec T cells, dec PTH, dec Ca
  • no thymic shadow
48
Q

IL-12 receptor deficiency

A
  • decreased Th1 response, AR
  • disseminated mycobacterial and fungal infections, may present after administration of BCG vaccine
  • decreased IFN gamma
49
Q

AD Hyper-IgE syndrome (Job syndrome)

A
  • deficiency of Th17 cells due to STAT3 mutation –> impaired recruitment of neutrophils to sites of infection
  • FATED: coarse Facies, staph Abscesses, retained Primary teeth, inc IgE, Dermatologic problems
50
Q

Chronic mucocutaneous candidiasis

A
  • T cell dysfunction (many causes)
  • non invasive candida infections of the skin and mucous membranes
  • absent in vitro T cell proliferation or cutaneous reaction in response to candida antigens
51
Q

SCID

A
  • several types, most important is adenosine deaminase deficiency (AR)
  • adenosine accumulation is toxic to T and B cells
  • FTT, chronic diarrhea, thrush
  • recurrent bacterial, fungal, viral and protozoal infections
  • no thymic shadow, no germinal centers
  • DO NOT GIVE LIVE VACCINES
  • treat with BM transplant
52
Q

Ataxia-telangiectasia

A
  • defects in ATM gene – DNA double strand breaks – cell cycle arrest
  • triad: Ataxia (cerebellar defects), spider Angiomas, IgA deficiency
  • inc AFP, dec IgA, IgG, IgE, lymphopenia and cerebellar atrophy
53
Q

Hyper IgM syndrome

A
  • defective CD40L on Th cells –> X linked recessive class switching defect
  • severe pyogenic infections early in life, opportunistic infections
  • inc IgM with all other lines down
54
Q

Wiskott-Aldrich Syndrome

A
  • WATER
  • Wiskott Aldrich: Thrombocytopenic purpura, Eczema, Recurrent infections
  • inc risk of autoimmune disease and malignancy
  • dec to normal IgM and IgG
  • inc IgE and IgA
55
Q

Leukocyte adhesion deficiency

A
  • defect in LFA-1 integrin (CD18) protein on phagocytes, impaired migration and chemotaxis, AR
  • recurrent bacterial skin and mucosal infections, no pus formation, impaired wound healing, delayed umbilical cord separation
  • inc neutrophils, none at infection site
56
Q

Chediak-Higashi syndrome

A
  • defect in lysosomal trafficking regulator gene (LYST)
  • microtubule disfunction in phagosome-lysosome fusion, AR
  • recurrent pyogenic infections by staph and strep, peripheral neuropathy, progressive neurodegeneration, infiltrative lymphohistocytosis
  • giant granules in neutrophils and platelets, pancytopenia and mild coag defects
57
Q

Chronic Granulomatous Disease

A
  • defect in NADPH oxidase, no respiratory burst and dec ROS
  • X linked recessive
  • inc susceptibility to cat + organisms
  • abnormal dihydrorhodamine (flow cytometry) test
  • nitroblue tetrazolium due reduction test is negative (neutrophils will not turn blue)
58
Q

acute transplant rejection

A
  • CTLs activated against donor MHCs + humoral Abs develop after transplant
  • leads to vasculitis of the graft vessels with dense interstitial lymphocytic infiltrate
  • prevent/reverse with immunosuppressants (calcineurin inhibitors)
59
Q

chronic transplant rejection

A

humoral and cellular, recipient T cells react against donor antigens presented on donor MHC

  • irreversible damage
  • heart - atherosclerosis
  • lungs - inflammation/fibrosis of small bronchioles (bronchiolitis obliterans)
  • liver - vanishing bile ducts
  • kidney - vascular fibrosis, glomerulopathy, loss of glomeruli, atrophy
60
Q

graft v. host disease

A

graft T cells proliferate in the immunocompromised host and reject host cells with “foreign” proteins –> severe organ dysfunction

  • rash, jaundice, diarrhea, HSmegaly
  • usually in BM and liver transplants
  • potentially beneficial for BM transplant
61
Q

cyclosporine

A
  • calcineurin inhibitor that blocks cyclophilin
  • blocks T cell activation by preventing IL-2 trascription
  • used for rejection ppx, psoriasis, RA
  • tox: nephrotox, HTN, HLD, hyperglycemia, tremor, hirsuitism, gingival hyperplasia
62
Q

tacrolimus

A
  • calcineurin inhibitor, binds FK506 binding protein (FKBP)
  • blocks T cell activation by preventing IL-2 transcription
  • used for rejection ppx
  • tox: similar to cyclosporine, inc risk of diabetes and neurotoxicity, no gingival hyperplasia or hirsuitism
63
Q

sirolimus

A

aka rapamycin

  • mTOR inhibitor, binds FKBP
  • blocks T cell activation and B cell differentiation by preventing IL-2 signal transduction
  • used in kidney transplant rejection prophylaxis
  • tox: anemia, thrombocytopenia, leukopenia, insulin resistance, HLD, non-nephrotoxic
  • kidney “sir”vives
64
Q

basiliximab

A
  • monoclonal Ab blocks IL-2R
  • kidney transplant rejection ppx
  • tox: edema, HTN, tremor
65
Q

azathioprine

A
  • antimetabolite precursor of 6-mercaptopurine
  • inhibits lymphocyte proliferation by blocking nucleotide synthesis
  • used in rejection ppx, RA, crohns, glomerulonephritis, and other AI conditions
  • tox: leukopenia, anemia, thrombocytopenia
  • degraded by xanthine oxidase, tox increases with allopurinol
66
Q

glucocorticoids

A
  • inhibits NFkB
  • suppress both B and T cell function by dec transcription of many cytokines
  • used in rejection ppx, many AI d/o, inflammation
  • tox: hyperglycemia, osteoporosis, central obesity, muscle breakdown, psychosis, acne, HTN, cataracts, peptic ulcers
67
Q

alemtuzumab

A
  • targets CD52
  • used in CLL
  • “alymtuzumab” used in chronic Lymphocytic leukemia
68
Q

bevacizumab

A
  • VEGF Ab

- used in colorectal cancer, RCC

69
Q

cetuximab

A
  • EGFR Ab

- used in stage IV colorectal cancer, head and neck cancer

70
Q

rituximab

A
  • CD20 Ab

- used in B cell non-Hodgkin lymphoma, RA and ITP

71
Q

trastuzumab

A
  • HER2/neu Ab

- used in breast cancer and gastric cancer

72
Q

infliximab, adalimumab

A
  • TNF alpha antibody

- used in IBD, rheumatoid arthritis, ank spon, psoriasis

73
Q

natalizumab

A
  • alpha 4 integrin Ab
  • used in MS and crohns
  • alpha 4 is used in leukocyte adhesion
  • risk of PML in pts with JC virus
74
Q

abciximab

A
  • glycoprotein IIb/IIIa Ab

- anti-platelet agent for prevention of ischemic complications in pts undergoing PCI

75
Q

denosumab

A

RANKL Ab

- used for osteoporosis, inhibits osteoclast maturation

Step 1 Flash Cards (36 decks)

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