Pathology EMQ

Name: Vrijraj Rathod

Theme: Defects in Primary Immunodeficiency

 

OPTION LIST

 

A

Severe combined immunodeficiency (SCID)

I

Chediak-Higashi Syndrome

B

Reticular dysgenesis

J

Ataxia telangiectasia

C

Selective IgA deficency

K

Wiskott-Aldrich Syndrome

D

DiGeorge’s Syndrome

L

Intereferon- Gamma- receptor defect

E

Kostmann syndrome

M

Bare lymphocyte syndrome

F

X-Linked Agammaglobulinaemia

N

Job Syndrome

G

Selective IgA deficency

O

Common variable immune deficiency

H

Hyper IgM sydrome

P

Leukocyte Adhesion Deficiency

 

For each scenario below, choose the most appropriate answer from the list above. Each option may be used once, more than once or not at all.

 

1.  Disease associated with thrombocytopenia, eczema, fatal infections and lymphoid malignancy

 

 

2. Defect in the gene encoding for CD40 Ligand on B Cells

 

 

3. Defect in Bruton’s tyrosine kinase.

 

 

4. Low levels of most immunoglobulin isotypes

 

 

5. Defect in MHC class II gene promoter

 

 

ANSWERS

1.  K

2. H

3.  F

4. O

5. M