Immuno Flashcards
Site of B-cell localization and proliferation
Follicle
Underdeveloped in DiGeorge
Paracortex
Site of T-cell differentiation and maturation
Thymus
HLA associated with Hemochromatosis
A3
HLA associated with Addison disease, myasthenia gravis, Graves
disease
B8
HLA associated with Psoriatic arthritis, Ankylosing spondylitis, IBD-associated arthritis, Reactive arthritis
B27
HLA associated with Celiac disease
DQ 2 and 8
HLA associated with Multiple sclerosis, hay fever, SLE, Goodpasture syndrome
DR2
HLA associated withDiabetes mellitus type 1, SLE, Graves disease, Hashimoto thyroiditis, Addison disease
DR3
HLA associated with Rheumatoid arthritis, diabetes mellitus type 1, Addison disease
DR4
IPEX
(Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked) syndrome
-Genetic deficiency of FOXP3 (defective Regulatory T cells) autoimmunity
Characterized by enteropathy, endocrinopathy, nail dystrophy, dermatitis, and/or other autoimmune dermatologic conditions. Associated with diabetes in male infants
C3 deficiency
↑ risk of severe, recurrent pyogenic sinus and respiratory tract infections: incr susceptibility to T3HS
CD55 deficiency
Also called decay-accelerating factor (DAF) deficiency.
Causes complement-mediated lysis of RBCs and paroxysmal nocturnal hemoglobinuria
T1 HS
Anaphylaxis
Test: skin test or blood test (ELISA) for allergenspecific
IgE
T2 HS
Autoimmune-hemolytic anemia Immune thrombocytopenic purpura Transfusion reactions Hemolytic disease of the newborn Goodpasture syndrome Rheumatic fever Hyperacute transplant rejection Myasthenia gravis Graves disease
T3 HS
SLE
Polyarteritis nodosa
Poststreptococcal glomerulonephritis
T4 HS
Type 1 diabetes mellitus Contact dermatitis (eg, poison ivy, nickel allergy) Graft-versus-host disease PPD
Febrile nonhemolytic transfusion reaction
T2HS
Host antibodies against donor HLA antigens and WBC
Within 1–6 hours
Acute hemolytic transfusion reaction
T2HS Intravascular hemolysis (ABO blood group incompatibility) or extravascular hemolysis (host antibody reaction against foreign antigen on donor RBCs)
Transfusion-related acute lung injury
Donor anti-leukocyte antibodies against recipient neutrophils and pulmonary endothelial cells
Anti-ACh receptor
Myasthenia gravis
Anti-glomerular basement membrane
Goodpasture syndrome
Anti-β2 glycoprotein
Antiphospholipid syndrome
Anticardiolipin, lupus anticoagulant
SLE, antiphospholipid syndrome
Anticentromere
Limited scleroderma (CREST syndrome)
Anti-desmoglein (anti-desmosome)
Pemphigus vulgaris
Anti-glutamic acid decarboxylase, islet cell
cytoplasmic antibodies
T1 DM
Anti-hemidesmosome
Bullous pemphigoid
Antisynthetase (eg, anti-Jo-1), anti-SRP, antihelicase (anti-Mi-2)
Polymyositis, dermatomyositis
Antimicrosomal, antithyroglobulin, anti-thyroid peroxidase
Hashimoto thyroiditis
Antimitochondrial
1° biliary cirrhosis
Antiparietal cell, anti-intrinsic factor
Pernicious anemia
Antiphospholipase A2 receptor
1° membranous nephropathy
Anti-Scl-70 (anti-DNA topoisomerase I)
Scleroderma (diffuse)
Anti-smooth muscle
Autoimmune hepatitis type 1
Anti-SSA, anti-SSB (anti-Ro, anti-La)
Sjögren syndrome
Anti-TSH receptor
Graves disease
Anti-presynaptic voltage-gated calcium channel
Lambert-Eaton myasthenic syndrome
IgA anti-endomysial, IgA anti-tissue transglutaminase
Celiac disease
MPO-ANCA/p-ANCA
Microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), ulcerative colitis
PR3-ANCA/c-ANCA
Granulomatosis with polyangiitis (Wegener)
Rheumatoid factor (IgM antibody against IgG Fc region), anti-CCP (more specific)
Rheumatoid arthritis
Antinuclear (ANA)
Nonspecific screening antibody, often associated with SLE
Anti-dsDNA, anti-Smith Anti-histone
SLE, drug induced SLE
Anti-U1 RNP (ribonucleoprotein)
Mixed connective tissue disease
X-linked (Bruton) agammaglobulinemia
XR; B-cell disorder
Defect in BTK, a tyrosine kinase gene: no B-cell maturation
Dear Ig of all classes; Absent/scanty lymph nodes and tonsils
Selective IgA deficiency
Decr IgA
Incr susceptibility to giardiasis
Airway and GI infections, Autoimmune disease, Atopy, Anaphylaxis to IgA-containing products
Common variable immunodeficiency
Defect in B-cell differentiation
DiGeorge syndrome
- Thymic aplasia
- 22q11 deletion; failure to develop 3rd and 4th pharyngeal pouches: absent thymus and parathyroids
- Tetany (hypocalcemia), recurrent viral/fungal infections (T-cell deficiency), conotruncal abnormalities
IL-12 receptor deficiency
↓ Th1 response, dear IFN-gamma
Autosomal dominant hyper-IgE syndrome
Job Syndrome
Deficiency of Th17 cells due to STAT3 mutation -> impaired recruitment of neutrophils to sites of infection
Incr IgE, decr IFN-gamma
Ataxia-telangiectasia
Cerebellar defects (Ataxia), spider Angiomas (telangiectasia), IgA deficiency
↑ AFP, risk of lymphoma and leukemia
Decr IgA, IgG and IgE
Hyper-IgM syndrome
Defective CD40L on Th cells; no class switching
Wiskott-Aldrich syndrome
Mutation in WASp gene; leukocytes and platelets unable to reorganize actin cytoskeleton; defective antigen presentation
↑ IgE and IgA
Leukocyte adhesion deficiency (type 1)
Defect in LFA-1 integrin(CD18) protein on phagocytes; impaired migration and chemotaxis; autosomal recessive
↑ neutrophils
Chédiak-Higashi syndrome
-Defect in lysosomal trafficking regulator gene (LYST)
Microtubule dysfunction in phagosome lysosome fusion; autosomal recessive
-Recurrent pyogenic infections by staphylococci and streptococci, partial albinism, peripheral neuropathy, progressive
neurodegeneration, infiltrative lymphohistiocytosis.
Chronic granulomatous disease diagnosis
Abnormal dihydrorhodamine (flow cytometry) test Nitroblue tetrazolium dye reduction test (obsolete) fails to turn blue
What is CREST?
Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactyly, Telangiectasia
Role of CD8 cells
Kill virally infected cells
Drugs with zero order kinetics
Phenytoin, ethanol, salicylates, fluoxetine, omeprazole, and cisplatin
The Pygmalion effect
Phenomenon of groups altering their outcomes to meet external expectations
Chapman’s point for the bladder
Periumbilical
SCID
IL2-R or ADA deficiency
TtF, diarrhea, thrush, all infection type
No thymic shadow