immunology Flashcards
HLA-A3
hemochromatosis
HLA-B27
seronegative arthropathies
HLA-DQ2/DQ8
celiac dz
HLA-DR2
MS, hay fever, SLE, goodpastures
HLA-DR3
T1DM, SLE, graves, hashimotos
HLA-DR4
RA, T1DM
HLA-DR5
pernicious anemia, hashimotos
type I HSR
IgE -> mast cell and basophil degranulation -> vasodilation of postcapillary venules -> delayed response from arachidonic acid metabolites (leukotrienes)
type II HSR
IgM, IgG bind to Ag on enemy cell -> cytotoxic killing via opsonization, complement + Fc receptor-mediated, and Ab-mediated cellular dysfxn. coombs test. acute transfusion rxns, AIHA, GBS, graves, MG, rheumatic fever, etc.
type III HSR
Ag, Ab, and complement get stuck together -> neutrophils release lysosomal enzymes. serum sickness and arthus rxn are specific types. SLE, post-strep glomerulonephritis, etc.
type IV HSR
NOT Ab-relatedd. delayed. T cells bind Ag -> cytokines -> macrophage activation. transplant rejection, TB skin test, touching (contact dermatitis). MS.
febrile nonhemolytic transfusion rxn
type II HSR. host Abs against donor HLA and WBCs -> fever, h/a, chills, flushing
acute hemolytic transfusion rxn
type II HSR. ABO incompatibility -> intravascular hemolysis, host Ab rxn w/foreign Ags -> extravascular hemolysis -> fever, hypotension, tachypnea, tachycardia, flank pain, hemoglobinuria (intravascular), jaundice (extravascular).
why does complement deficiency increase risk of SLE?
b/c complement is necessary to remove Ag/Ab complexes
anti-basement membrane
goodpastures
anticardiolipin, lupus anticoagulant
SLE, antiphospholipid
anticentromere
limited scleroderma (CREST)
anti-desmosome, anti-desmoglein
pemphigus vulgaris
anti-smith
SLE
anti-glutamic acid decarboxylase (GAD-65)
T1DM
antihemidesmosome
bullous pemphigoid
anti-histone
drug-induced lupus
anti-Jo-1, anti-SRP, anti-Mi-2
polymyositis, dermatomyositis
antimicrosomal, antithyroglobulin
hashimotos
antimitochondrial
PBS
antinuclear Abs
SLE, nonspecific
antiparietal cell
pernicious anemia
anti-Scl-70, anti-DNA topoisomerase I
scleroderma (diffuse)
anti-SSA, anti-SSB (anti-Ro, anti-La)
sjogrens
anti-smooth muscle
autoimmune hepatitis
anti-TSH
graves
anti-U1 RNP (ribonucleoprotein)
mixed connective tissue dz
IgA anti-endomysial, IgA anti-tissue transglutaminase
celiac
MPO-ANCA/p-ANCA
microscopic polyangiitis, eosinophilis granulomatosis w/polyangiitis (churg-strauss)
PR3-ANCA/c-ANCA
granulomatosis w/polyangiitis (wegener)
RF, anti-CCP
RA. RF = IgM against IgG Gc region. anti-CCP = more specific
immunodeficiency that -> giardia, bacterial, enteroviral infxns after 6 mos
bruton’s agammaglobulinemia. no BTK -> no B cell maturation, so no plasma cells. absent/scanty lymph nodes
immunodeficiency that -> recurrent mucosal infxns, esp. viral
selective IgA def. usually asymptomatic.
immunodef. that -> bacterial, enterovirus, and giardia infxns acquired later
common variable immunodef. many causes (e.g. B cell or helper T cell defect) -> B cell maturation failure -> dec. plasma cells, immunoglobulins. inc. risk of autoimmune dz, bronchiectasis, lymphoma, sinopumonary infxns.
IL-12 def.
dec. Th1 response. AR. -> disseminated mycobacterial and fungal infxns. may present after BCG vaccine. dec. IFN-gamma
hyper-IgE syndrome
AD. job syndrome. STAT3 mutation -> Th17 def. -> impaired neutophil recruitment. course facies, cold (non-inflamed) staph abscesses, retained primary teeth, inc. IgE, dermatologic problems (eczema). low IFN-gamma
no B cells ->
fungal + viral infxns
no T cells ->
bacterial + protozoal infxns
no IgA ->
enterovirus
no C5-9 ->
neisseria
no C1 inhibiter ->
hereditary angioedema (esp. periorbital) + mucosal surfaces
3 causes of SCID
cytokine receptor defects
adenosine deaminase deficiency
MHC II def. -> dec. CD4+ -> dec. T + B cells
ataxia-telangiectasia
ATM gene defect -> failure to repair dsDNA breaks -> cell cycle arrest -> cerebellar defects, spider angiomas, IgA def. + inc. AFP, low IgA, IgG, IgE. lymphopenia.
wiskott-aldrich
WAS mutation, XR -> T cells can’t reorganize actin cytoskeleton -> TTP, eczema, recurrent humoral + cellular infxns, inc risk of autoimmunity. low/nl IgG, IgM. inc. IgE, IgA. fewer, smaller plts.
leukocyte adhesion deficiency type 1
AR. defect in LFA-1 integrin CD18 subunit -> impaired migration + chemotaxis of phagocytes -> delayed cord separation, recurrent bacterial + mucosal infxns w/o pus.
chediak-higashi
AR defective lysosomal trafficking regulator gene (LYST). microtubule dysfxn -> lysosome + phagosome can’t fuse. -> recurrent pyogenic staph + strep infxns, partial albinism, long nerve neuropathy, infiltrative lymphohistiocytosis. giant granules in granulocytes + plts. pancytopenia (impaired cell division), mild coag defect (primary hemostasis problem)
MPO deficiency
defective conversion of H2)2 -> HOCL -> inc. candida. normal NBT test
cyclosporine
calcineurin inhibitor, binds cyclophilin. blocks T cell activation by preventing IL-2 transcription!! Tx for PPx transplant rejection, psoriasis, RA. nephrotoxic
tacrolimus
= FK506. calcineurin inhibitor, binds FKBP. blocks T-call activation by preventing IL-2 transcription! Tx: PPx transplant rejection. nephrotoxic! inc. DM, neurotox. no gingival hyperplasia or hirsuitism
sirolimus
= rapamycin. mTOR inhibitor; bind FKBP. blocks T cell activation/B cell differentiation by preventing response to IL-2! Tx: kidney tranplant recetion PPx. not nephrotoxic. cytopenias, insulin resistance, HL
daclizumab, basiliximab
Abs block IL–2R. Tx: kidney tranplant PPx. edema, HTN, tremor
azathioprine
antimetabolite precursor of 6-mercaptopurine. inhibits lymphocyte proliferation by blocking nucleotide synthesis. Tx: rejection PPx, autoimmune stuff. cytopenias. 6-MP is degraded by xanthine oxidase so allopurinol -> inc. toxicity.
glucocorticoids
inhibit NF-kappaB. suppress B and T cells by dec. cytokine transcription.
aldesleukin
= IL-2. use in renal cell CA, metastatic melanoma
epoetin alfa
= erythropoietin.
filgrastim
= G-CSF. use for bone marrow recovery
sargramostim
= GM-CSF. use for bone marrow recovery
IFN-alpha
use in chronic hep B + C, kaposi sarcoma, malignant melanoma.
IFN-beta
MS
IFN-gamma
CGD
romiplostim, eltrombopag
thrombocytopenia
oprelvekin
= IL-11. use in thrombocytopenia
alemtuzumab
targets CD52. use in CLL
bevacizumab
targets VEGF. use for colorectal CA, renal cell CA. neovascular macular degeneration.
cetuximab
targets EGFR. use in stage IV colorectal CA, head + neck CA
rituximab
targets CD20. use in H-cell NH lymphoma, CLL, RA, ITP
trastuzumab
targets HER2/neu
adalimumab, infliximab
targets soluble TNF-alpha. use in IBD, RA, AK, psoriasis
etanercept
decoy TNF-alpha receptor, not monoclonal Ab
eculizumab
targets complement protein C5. use in paroxysmal nocturnal hemoglobinuria
natalizumab
targets alpha4-integrin (WBC adhesion). use in MS, crohns. -> inc. risk of PML in pts w/JC virus.
abciximab
targets plt glycoproteins IIb/IIIa. use to prevent ischemic complications during vascular cath.
denosumab
targets RANKL. use in osteoporosis to inhibit osteoclast maturation.
digoxin immune Fab
antidote for dig toxicity
omalizumap
targets IgE. use for allergic asthma: prevents IgE binding to Fc-epsilon-RI
palivizumap
targets RSV F protein. use as RSV PPx in high risk babies