Pathology EMQ template.

Name: Samiha Hayek

Theme: Immune Response to Infection-2

 

OPTION LIST

 

A	Reticular dysgenesis	I	Bare Lymphocyte Syndrome -Type 2
B	Bruton’s X-linked hypogammaglobulinaemia	J	Bare Lymphocyte Syndrome – Type 1
C	Di George Syndrome	K
D	Kostmann’s syndrome	L	Chronic Granulomatous Disease
E	IFN gamma receptor deficiency	M	Selective IgA deficiency
F	Hyper IgM syndrome	N	Severe Combined Immunodeficiency
G	C6 deficiency	O	Mannose Binding Lectin deficiency
H	Cyclic neutropaenia	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.    A phagocyte disorder characterised by a deficiency in one of the components of NADPH oxidase.

 

 

2.  Recurrent infections with no neutrophils on FBC.

 

 

3.  A 3 month year old presents with recurrent bacterial infections     and a failure to thrive. The baby has a very low T cell count and normal B cell count.

 

 

4.  Absent expression of MHC class II molecules.

 

 

5.  Associated with a defective B cell tyrosine kinase gene.

 

 

 

ANSWERS

1.

2.

3.

4.

5.