Immunodeficiency and Immunomodulation

Question 1

Autoantibody in DM (autoimmune form)

Answer 1

Antiinsulin receptor

Question 2

Autoantibody in Goodpasture’s syndrome

Answer 2

Antibasement membrane

Question 3

Autoantibody in Grave’s disease

Answer 3

Thyroid stimulating immunoglobulins (anti TSH receptor)

Question 4

Autoantibody in Hashimoto’s thyroiditis

Answer 4

Antithyroglobulin

Question 5

Autoantibody in RA

Answer 5

Anti-EBV related Ag

Question 6

Autoantibody in Sjogren’s syndrome

Answer 6

Anti-salivary duct epithelium

Anti-SSA

Anti-SSB

Question 7

Cytokines secreted by Th₁ cells

Answer 7

Production/secretion of IL-2 and TNF

Question 8

Cytokines secreted by Th₂ cells

Answer 8

IL-4

IL-5

IL-6

IL-10

Question 9

A. CD40ligand
B. FoxP3
C. TAP
D. IL-2 receptor 𝜸 (gamma) subunit
E. NADPH oxidase

Answer 9

D. IL-2 receptor 𝜸 (gamma) subunit

Question 10

What features best differentiates CVID from other immunodeficiencies?

Answer 10

• Decreased IgG/M/A in absence of other defined immunodeficiencies
• Typically presents later in life than other primary immunodeficiencies (~20-40 y/o)

Question 11

A. IL-12 receptor deficiency
B. Ataxia-telangiectasia
C. Hyper IgE syndrome
D. Wiskott-Aldrich syndrome
E. ADA SCID

Answer 11

A. IL-12 receptor deficiency

Question 12

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

Answer 12

A. X-linked SCID and

E. ADA SCID

Question 13

A. ATM gene
B. STAT-3 gene
C. CD40 ligand gene
D. STAT-1 gene

Answer 13

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

Question 14

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

Answer 14

A. RAG SCID, B. X-linked agammaglobulinemia, C. ADA SCID

Question 15

Answer 15

Pneumocystis jerovecii pneumonia and elevated IgM

Hyper IgM syndrome (usually x-linked) - CD40 defect prevents class switching

Question 16

Answer 16

D. IL-2 γ receptor

All immunoglobulins are reduced

X-linked - male

Various and numerous infections

A. Hyper IgM syndrome
B. Lack T_reg tends to present w/ autoimmune disease
C. Causes MHC class I deficiency
E. Chronic granulamtous disease

Question 17

Wiskott-Aldrich Syndrome

Answer 17

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

Question 18

Answer 18

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

Question 19

ATM deficiency

Answer 19

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)

Question 20

Answer 20

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

Question 21

Answer 21

D. X-linked SCID - lose CD4+ T cells

Bruton’s agammaglobulinemia results in absence (or almost complete absence) of the B cell line

Question 22

Azathioprine is convereted to 6-mercaptopurine - which is what type of analog?

Answer 22

Purine analog

Question 23

What drug-induced disorder represents a morjo limitation of the general use of most immunosuppresant drugs?

Answer 23

Lymphomas

Question 24

The immunosuppressive effect of glucocorticoids is primarily related to what MOA?

Answer 24

Inhibition of T cell-mediated production of IL-2

Question 25

Whic drug is a mTOR inhibitor?

A. Tacrolimus
B. Sirolimus
C. Cyclosporine
D. Prednisone
E. Azathioprine

Answer 25

B. Sirolimus

Question 26

Which of the following drugs can bind to and neutralize TNF alpha?

A. Muromonab CD3
B. Infliximab
C. Cetuximab
D. Trastuzumab
E. Bevacizumab

Answer 26

B. Infliximab

Question 27

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?

Answer 27

Cyclosporine

Question 28

Answer 28

Mycophenolate mofetil

Question 29

A. Aldesleukin
B. Vinblastine
C. Tacrolimus
D. INF-alpha 2a
E. Thalidomide

Answer 29

C. Tacrolimus

Question 30

MOA of muromonab-CD3

Answer 30

Drug neutralizes a surface protein receptor complex in T cells

Management of cytokine release syndrome is Tocilizumab (targets IL-6)

Question 31

Answer 31

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

Question 32

Answer 32

Lymphomas and infections - all other options are class specific

Question 33

MOA of sirolimus

Answer 33

Inhibits mTOR - prevents

Question 34

Answer 34

B. Infliximab

F. Ethanercept (fusion protein)

suffix -cept = fusion protein

Question 35

Answer 35

Question 36

Answer 36

D. Cyclosporine

Question 37

Effect of thalidomide

Answer 37

Inhibits angiogenesis - used to Tx some cancers

Question 38

Aldesleukin

Answer 38

Question 39

Immune thrombocytopenia purpura

Answer 39

Question 40

Mycophenolate mefotnil

Answer 40