Pathology EMQ template.

Name: Ali Hosin

Theme: Immune Response to Infection 2

 

OPTION LIST

 

A	Bare Lymphocyte Syndrome	I	Selective IgA deficiency
B	Bruton's X-linked hypogammaglobulinaemia	J	Severe Combined Immune Deficiency
C	C3 Complement Deficiency	K
D	DiGeorge Syndrome	L
E	Hyper IgM syndrome	M
F	IFN-γ receptor deficiency	N
G	Kostmann's Syndrome	O
H	Lectin Deficiency	P

 

 

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 24 year-old man, previously fit and well, diagnosed with lymphoma develops recurrent chest infections during his treatment.

 

2. A 1 year-old boy presents with 10 bacterial infections over the past year. Investigations reveal: CD4⁺ and CD8⁺ T cells present; B cells absent; IgG, IgA, IgM absent.

 

 

3. A 1 year-old boy with recurrent infections also has abnormal facial features. Investigations reveal: B cells normal; T cells low; IgA, IgG low. Chromosomal analysis reveals a mutation at 22q11

 

 

4. A 6-month old boy presents with failure to thrive. He was well until 3 months ago when he developed recurrent infections, diarrhoea and a skin rash. Investigations reveal: CD4⁺ and CD8⁺ T cells absent; B cells present; IgM present; IgG, IgA absent.

 

 

5. A 16 year-old boy presents to his GP with several months’ history of malaise. Blood cultures reveal infection with atypical Mycobacterium spp. All other investigations are normal. 

 

 

 

ANSWERS

1. H

2. B

3. D

4. J

5. F