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 and TNF
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
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
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
Wiskott-Aldrich Syndrome
Severe thrombocytopenia and progressive T cell dysfunction
Low IgM/G but elevated IgE/A
Pt’s do not make Ab’s to polysaccharide Ags
X-linked recessive disease caused by a defect in the gene encoding WASp - regulates actin polymerization - specifically affects Th17 cell line
TIE
Thrombocytopenia
Infection
IgE
A. IL-12 receptor deficiency
Most common defect that makes Pt’s hyper-susceptible to Mycobacteria tuberculosis
IL-12 ß1 deficiency results in Th0 NOT differentiating into Th1 cell line
ATM deficiency
Causes T-cell deficiency becaused ATM is important in somatic recombination in joining the VDJ segement of the TCR beta subunit (B cells are not as dependent on ATM but do require ATM during class switching)
C. Pyrin
FMF - deposition of amyloid A in kidneys (renal amylodiosis) causes death
Familial Mediterranean Fever (FMF; also termed recurrent polyserositis) is typically associated with recessive mutations in the MEFV gene that cause pyrin protein over-production. Pyrin is involved in inflammasome activation. Therefore, pyrin over-production results in chronic, increased production of IL-1 and other inflammatory cytokines
D. X-linked SCID - lose CD4+ T cells
Bruton’s agammaglobulinemia results in absence (or almost complete absence) of the B cell line
Azathioprine is convereted to 6-mercaptopurine - which is what type of analog?
Purine analog
What drug-induced disorder represents a morjo limitation of the general use of most immunosuppresant drugs?
Lymphomas
The immunosuppressive effect of glucocorticoids is primarily related to what MOA?
Inhibition of T cell-mediated production of IL-2
Whic drug is a mTOR inhibitor?
A. Tacrolimus
B. Sirolimus
C. Cyclosporine
D. Prednisone
E. Azathioprine
B. Sirolimus
Which of the following drugs can bind to and neutralize TNF alpha?
A. Muromonab CD3
B. Infliximab
C. Cetuximab
D. Trastuzumab
E. Bevacizumab
B. Infliximab
This drug is a macrolide antibiotic that works by binding to cyclophilin located in T cells, blocking the gene expression for IL 2 production. Which drug is it?
Cyclosporine
Mycophenolate mofetil
A. Aldesleukin
B. Vinblastine
C. Tacrolimus
D. INF-alpha 2a
E. Thalidomide
C. Tacrolimus
MOA of muromonab-CD3
Drug neutralizes a surface protein receptor complex in T cells
Management of cytokine release syndrome is Tocilizumab (targets IL-6)
Inhibition of macrophage-mediated production of IL-1
Inhibition of T cell-mediated production of IL-2
Direct lympholytic effect of cytotoxic T cells
Glucocorticoid inhibit immunophilins and phospholipase A2
Lymphomas and infections - all other options are class specific
MOA of sirolimus
Inhibits mTOR - prevents
B. Infliximab
F. Ethanercept (fusion protein)
suffix -cept = fusion protein
D. Cyclosporine
Effect of thalidomide
Inhibits angiogenesis - used to Tx some cancers
Aldesleukin
Immune thrombocytopenia purpura
Mycophenolate mefotnil
