Immunodeficiency and Immunomodulation Flashcards
Autoantibody in DM (autoimmune form)
Antiinsulin receptor
Autoantibody in Goodpasture’s syndrome
Antibasement membrane
Autoantibody in Grave’s disease
Thyroid stimulating immunoglobulins (anti TSH receptor)
Autoantibody in Hashimoto’s thyroiditis
Antithyroglobulin
Autoantibody in RA
Anti-EBV related Ag
Autoantibody in Sjogren’s syndrome
Anti-salivary duct epithelium
Anti-SSA
Anti-SSB
Cytokines secreted by Th1 cells
Production/secretion of IL-2,IFN-gamma
Cytokines secreted by Th2 cells
IL-4
IL-5
IL-6
IL-10
A. CD40ligand
B. FoxP3
C. TAP
D. IL-2 receptor 𝜸 (gamma) subunit
E. NADPH oxidase
D. IL-2 receptor 𝜸 (gamma) subunit (X-linked SCID)
What features best differentiates CVID from other immunodeficiencies?
- Decreased IgG/M/A in absence of other defined immunodeficiencies
- Typically presents later in life than other primary immunodeficiencies (~20-40 y/o)
A. IL-12 receptor deficiency
B. Ataxia-telangiectasia
C. Hyper IgE syndrome
D. Wiskott-Aldrich syndrome
E. ADA SCID
A. IL-12 receptor deficiency
In which disorder(s) would NK cells NOT be produced?
A. X-linked SCID
B. RAG SCID
C. Ataxia-telangiectasia
D. TAP deficiency
E. ADA SCID
A. X-linked SCID and
E. ADA SCID
A. ATM gene
B. STAT-3 gene
C. CD40 ligand gene
D. STAT-1 gene
B. STAT-3 gene
Autosomal dominant STAT-3 defects results in Hyper IgE syndrome -
impaired neutrophil recruitment to infection sites
The classic triad for hyper IgE syndrome is: i) eczema; ii)
recurrent C. albicans infections + “cold” Staph skin abscesses; and iii) increased serum IgE.
Differentiate from WAS - Hyper IgE is associated with recurrent fractures and retention of baby teeth
In which disorder(s) would circulating B cells NOT be produced?
A. RAG SCID
B. X-linked agammaglobulinemia
C. ADA SCID
D. X-linked SCID
A. RAG SCID, B. X-linked agammaglobulinemia, C. ADA SCID
Pneumocystis jerovecii pneumonia and elevated IgM
Hyper IgM syndrome (usually x-linked) - CD40 defect prevents class switching
D. IL-2 γ receptor = X-linked SCID
All immunoglobulins are reduced
X-linked - male
Various and numerous infections
A. Hyper IgM syndrome
B. Lack Treg tends to present w/ autoimmune disease
C. Causes MHC class I deficiency
E. Chronic granulamtous disease