Immunodeficiency and Immunomodulation Flashcards

1
Q

Autoantibody in DM (autoimmune form)

A

Antiinsulin receptor

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2
Q

Autoantibody in Goodpasture’s syndrome

A

Antibasement membrane

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3
Q

Autoantibody in Grave’s disease

A

Thyroid stimulating immunoglobulins (anti TSH receptor)

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4
Q

Autoantibody in Hashimoto’s thyroiditis

A

Antithyroglobulin

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5
Q

Autoantibody in RA

A

Anti-EBV related Ag

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6
Q

Autoantibody in Sjogren’s syndrome

A

Anti-salivary duct epithelium

Anti-SSA

Anti-SSB

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7
Q

Cytokines secreted by Th1 cells

A

Production/secretion of IL-2,IFN-gamma

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8
Q

Cytokines secreted by Th2 cells

A

IL-4

IL-5

IL-6

IL-10

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9
Q

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

A

D. IL-2 receptor 𝜸 (gamma) subunit (X-linked SCID)

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10
Q

What features best differentiates CVID from other immunodeficiencies?

A
  • Decreased IgG/M/A in absence of other defined immunodeficiencies
  • Typically presents later in life than other primary immunodeficiencies (~20-40 y/o)
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11
Q

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

A

A. IL-12 receptor deficiency

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12
Q

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

A. X-linked SCID and

E. ADA SCID

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13
Q

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

A

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

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14
Q

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

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

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15
Q
A

Pneumocystis jerovecii pneumonia and elevated IgM

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

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16
Q
A

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

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17
Q

Wiskott-Aldrich Syndrome

A

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

18
Q
A

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

19
Q

ATM deficiency

A

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)

20
Q
A

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

21
Q
A

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

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

22
Q

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

A

Purine analog

23
Q

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

A

Lymphomas

24
Q

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

A

Inhibition of T cell-mediated production of IL-2

25
Q

Whic drug is a mTOR inhibitor?

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

A

B. Sirolimus

26
Q

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

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

A

B. Infliximab

27
Q

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?

A

Cyclosporine

28
Q
A

Mycophenolate mofetil

29
Q

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

A

C. Tacrolimus

30
Q

MOA of muromonab-CD3

A

Drug neutralizes a surface protein receptor complex in T cells

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

31
Q
A

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

32
Q
A

Lymphomas and infections - all other options are class specific

33
Q

MOA of sirolimus

A

Inhibits mTOR - prevents

34
Q
A

B. Infliximab

F. Ethanercept (fusion protein)

suffix -cept = fusion protein

35
Q
A
36
Q
A

D. Cyclosporine

37
Q

Effect of thalidomide

A

Inhibits angiogenesis - used to Tx some cancers

38
Q

Aldesleukin

A

Used to Tx renal carcinoma and malignant melanoma

39
Q

Immune thrombocytopenia purpura

A
40
Q

Mycophenolate mofetil

A