Name: Andrew O'Brien

Theme: Paediatric Haematology

OPTION LIST

A	Glucose-6 phosphate deficiency	I	Sickle cell trait
B	Sickle cell disease	J	Haemophilia B
C	Hereditary Spherocytosis	K	von Willebrand’s disease
D	Autoimmune haemolytic anaemia	L	Haemophilia A
E	Haemolytic disease of the newborn	M	Thrombotic thrombocytopenic purpura
F	Henoch-Schönlein Syndrome	N	Hereditary stomatocytosis
G	Polycythaemia	O	Polycythaemia vera
H	Β-Thalassaemia	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 new-born male presents with jaundice. A blood film reveals the presence of spherocytes and reticulocytes. Both serum bilirubin and urinary urobiligen are raised. A Coombs test yields a negative result.

2. A mother of Greek origin is worried about her 2 month old son. He has developed sudden pallor and jaundice. Irregularly contracted cells are seen on the blood film.

3. A three year old boy presents with a purpuric rash across the legs and buttocks. Two weeks previously you had seen the child about a chest infection. Further examination reveals abdominal pain and haematuria. Serum IgA levels are raised.

4. A two year old Afro-Caribbean boy presents to A&E with ‘hand-and-foot’ syndrome. You discover that he has never had a Guthrie Spot test.

5. A mother gives birth to twins. One appears to be pale and is in fact anaemic. This is due to twin-to-twin transfusion. What haematological abnormality is the other twin most likely to have?

ANSWERS

1. C

2. A

3. F

4. B

5. G