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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
Bruton's agammaglobulinemia
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
Selective IgA def
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
CVID
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
Digeorge
22q11, failure of 3rd and 4th pouches | Tetany, recurrent viral/fungal infxn, congenital heart/vessel dz
29
IL-12 R deficiency
decr Th1 | Disseminated mycobacterial ifxns
30
Hyper IgE (Job's)
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
Chronic mucocutaneous candidiasis
T cell dysfunction | candida infxn of skin and mucous membranes
32
SCID
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
Ataxia-telangiectasia
defects in ATM gene (DNA repair) Triad: cerebellar defects, spirder angiomas, IgA deficiency Incr AFP
34
Hyper-IgM
Most commonly, defect in CD40L --> no class switching Severe pyogenic infxns early in life incr IgM, decr others
35
Wiskott-aldrich
XLR defect in WAS gene --> T Cells unable to reorganize actin cytoskeleton Thrombocytopenic purpura, Infections, Eczema Incr IgE/IgA, decr IgM, thrombocytopenia
36
Leukocytes adhesion deficiency
Defect in LFA-1 integrin (CD18) on phagocytes | Recurrent bacterial infections, absent pus formation, delayed separation of umbilical cord. Neutrophilia
37
Chediak-Higashi syndrome
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
CGD
Lack of NADPH oxidase --> decr superoxide and absent respiratory burst. incr catalase-positive infxns (Staph, E Coli, aspergillus) Abnormal DHR flow cytometry
39
Encapsulated Bacteria
SHINE SKiS | Strep pneumo, Hib, Neisseria meningitidis, E Coli, Salmonella, Klebsiella, GBS
40
Hyperacute rejection
minutes Type II (Ab-mediated). preformed anti-donor Abs occludes graft vessels --> ischemia/necrosis
41
Acute rejection
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
Chronic rejection
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
GVHD
timing varies Grafted immunocompetent T cells proliferate in host and reject cells maculopapular rash, jaundice, HSM, diarrhea.

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