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Flashcards in Immunology Deck (43):
1

C1 esterase inhibitor deficiency

Hereditary angioedema.
ACEi contraindicated

2

C3 deficiency

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

3

C5-C9 deficiencies

Recurrent Neisseria bacteremia

4

DAF (GPI anchored enzyme) deficiency

complement-mediated lysis of RBCs and PNH

5

IL-1

pyrogen
Activates endothelium to express adhesion molecules

6

IL-2

Stimulates T cells (H, C, Reg)

7

IL-4

TH2 cells (eos), IgE

8

IL-5

TH2 cells, IgA, eos

9

IL-12

TH1 cells (MPHages)

10

IL-6

Pyrogen, acute phase proteins

11

IL-8

chemotaxis of PMNs

12

TNF-alpha

septic shock. activates endothelium. leukocyte recruitment, vascular leak

13

IL-3

Bone Marrow. G-CSF

14

IFN-g

macrophages, TH1
Suppresses TH2
antiviral, antitumor
granuloma

15

IL-10

Treg
Inhibits activated T cells

16

TGF-b

Treg
Inhibits activated T cells

17

Type I Hypersensitivity


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

18

Type II Hypersensitivity

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

Type III Hypersensitivity

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

Type IV Hypersensitivity

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

21

Allergic transfusion rxn

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

22

Anaphylactic transfusion rxn

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

23

FNHTR

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

24

acute hemolytic transfusion rxn

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.