Immunology Flashcards

1
Q

C1 esterase inhibitor deficiency

A

Hereditary angioedema.

ACEi contraindicated

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2
Q

C3 deficiency

A

Severe, recurrent pyogenic sinus respiratory tract infections. Incr susceptibility to type III hypersensitivity rns

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3
Q

C5-C9 deficiencies

A

Recurrent Neisseria bacteremia

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4
Q

DAF (GPI anchored enzyme) deficiency

A

complement-mediated lysis of RBCs and PNH

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5
Q

IL-1

A

pyrogen

Activates endothelium to express adhesion molecules

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6
Q

IL-2

A

Stimulates T cells (H, C, Reg)

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7
Q

IL-4

A

TH2 cells (eos), IgE

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8
Q

IL-5

A

TH2 cells, IgA, eos

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9
Q

IL-12

A

TH1 cells (MPHages)

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10
Q

IL-6

A

Pyrogen, acute phase proteins

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11
Q

IL-8

A

chemotaxis of PMNs

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12
Q

TNF-alpha

A

septic shock. activates endothelium. leukocyte recruitment, vascular leak

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13
Q

IL-3

A

Bone Marrow. G-CSF

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14
Q

IFN-g

A

macrophages, TH1
Suppresses TH2
antiviral, antitumor
granuloma

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15
Q

IL-10

A

Treg

Inhibits activated T cells

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16
Q

TGF-b

A

Treg

Inhibits activated T cells

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17
Q

Type I Hypersensitivity

A

IgE cross-linking on presensitized mast cells/basophills –> histamine etc
Test: skin test for IgE
Ex: anaphylaxis, atopy

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18
Q

Type II Hypersensitivity

A

Cytotoxic, antibody-mediated (IgM, IgG)
1. Opsonize –> phagocytosis or complement
2. complement-lysis (MAC)
3. ADCC (NK cells)
Test: Coombs’
Ex: AIHA, Pernicious anemia, ITP, Erythroblastosis fetalis, Acute hemolytic transfusion rxn, Rhematic fever, Goodpasture’s, Bullous pemphigoid, pemphigoid vulgaris

19
Q

Type III Hypersensitivity

A

Immune complex –> PMNs
Serum sickness
Arthus rxn (local response –> edema, necrosis, complement
Test: immunofluorescent staining
ex: SLE, polyarteritis nodosa, PSGN, Serum sickness, Arthus rxn (tetanus vaccine)

20
Q

Type IV Hypersensitivity

A

Delayed (T cell –> MPhage)
No Antibodies
Test: patch test, PPD
Ex: MS, GBS, GVHD, PPD, contact dermatitis

21
Q

Allergic transfusion rxn

A

Type I against plasma proteins
urticaria pruritus, wheezing, fever
Tx: antihistamines

22
Q

Anaphylactic transfusion rxn

A

severe.
IgA-deficient Pts (must receive blood w/o IgA)
Bronchospasm, hypotension, shock

23
Q

FNHTR

A

Type II hypersensitivity. Host Abs against donor HLA antigens and leukocytes
Fever, headache, chills, flushing

24
Q

acute hemolytic transfusion rxn

A
type II hpersensitivity.
Intravascular hemolysis (ABO incompatibility) or extravascular hemolysis (host Ab rxn to antigen on donor RBCs
fever, hypotension, tachypnea, tachycardia, hemoglobinemia, jaundice
25
Q

Bruton’s agammaglobulinemia

A

XLR Defect in BTK (tyrosine kinase) –> no B cell maturation
Recurrent bacterial infections after 6 months (maternal IgG)
Normal pro-B, decr Bcells, dec Ig of all types
Tx: IVIG

26
Q

Selective IgA def

A

unknown cause
Majority asymptomatic, but can have sinopulmonary, GI (giardia!), autoimmune dz, anaphylaxis to blood products
Dx: IgA <7 w normal IgG, M. False-positive BhCG

27
Q

CVID

A

Defect in B cell maturation (many causes)
can acquire in 20s-30s
incr risk of autoimmune dz, lymphoma, sinopulmonary infxn
normal # B cells but decr plasma cells and Ig

28
Q

Digeorge

A

22q11, failure of 3rd and 4th pouches

Tetany, recurrent viral/fungal infxn, congenital heart/vessel dz

29
Q

IL-12 R deficiency

A

decr Th1

Disseminated mycobacterial ifxns

30
Q

Hyper IgE (Job’s)

A

Th1 cells fail to produce IFNg –> inability of PMNs to respond to chemotactic stimuli
coarse facies, noninflammed staph abscesses, retained primary Teeth, incr IgE, Derm problems (eczema)

31
Q

Chronic mucocutaneous candidiasis

A

T cell dysfunction

candida infxn of skin and mucous membranes

32
Q

SCID

A

multiple types: defective IL2 R (XLR), adenosine deaminase deficiency
FTT, chronic diarrhea, thrush, absence of thymic shadow, germinal bx, and BCells, DECR TRECs (tcell recombinant excision circles)
Tx: BMT (no rejection)

33
Q

Ataxia-telangiectasia

A

defects in ATM gene (DNA repair)
Triad: cerebellar defects, spirder angiomas, IgA deficiency
Incr AFP

34
Q

Hyper-IgM

A

Most commonly, defect in CD40L –> no class switching
Severe pyogenic infxns early in life
incr IgM, decr others

35
Q

Wiskott-aldrich

A

XLR defect in WAS gene –> T Cells unable to reorganize actin cytoskeleton
Thrombocytopenic purpura, Infections, Eczema
Incr IgE/IgA, decr IgM, thrombocytopenia

36
Q

Leukocytes adhesion deficiency

A

Defect in LFA-1 integrin (CD18) on phagocytes

Recurrent bacterial infections, absent pus formation, delayed separation of umbilical cord. Neutrophilia

37
Q

Chediak-Higashi syndrome

A

AR, defect in LYST. Microtubule dysfunction in phagosome/lysosome fusion
Recurrent pyogenic infections by staph/strep, partial albinism, peripheral neuropathy
Giant granules in PMNs

38
Q

CGD

A

Lack of NADPH oxidase –> decr superoxide and absent respiratory burst. incr catalase-positive infxns (Staph, E Coli, aspergillus)
Abnormal DHR flow cytometry

39
Q

Encapsulated Bacteria

A

SHINE SKiS

Strep pneumo, Hib, Neisseria meningitidis, E Coli, Salmonella, Klebsiella, GBS

40
Q

Hyperacute rejection

A

minutes
Type II (Ab-mediated). preformed anti-donor Abs
occludes graft vessels –> ischemia/necrosis

41
Q

Acute rejection

A

weeks
Cell mediated 2/2 CTLs reacting against foreing MHCs
Vasculitis of graft vessels with dense interstitial lymphocytic infiltrate
reversible with immunosuppressants (e.g. cyclosporine, muromonab-CD3)

42
Q

Chronic rejection

A

months to years
Class I MHC (non-self) is perceived by CTLs as Class I MHC (self) presenting non-self antigen
Irreversible T-cell antibody-mediated vascular damage (obliterative vascular fibrosis, bronchiolitis obliterans –lung), fibrosis of graft tissues

43
Q

GVHD

A

timing varies
Grafted immunocompetent T cells proliferate in host and reject cells
maculopapular rash, jaundice, HSM, diarrhea.