Name:

Candidate number:

Theme: Paediatric Haematology

 

OPTION LIST

A	Alpha Thalassaemia Trait	I	Haemophilia B
B	Autoimmune Haemolytic Anaemia	J	Hereditary Elliptocytosis
C	Beta Thalassaemia Major	K	Hereditary Spherocytosis
D	Beta Thalassaemia Trait	L	Iron Deficiency Anaemia
E	Glucose-6-phosphate dehydrogenase (G6PD) deficiency	M	Pyruvate Kinase deficiency
F	Haemoglobin Barts Hydrops Fetails	N	Sickle Cell Anaemia
G	Haemolytic Uraemic Syndrome	O	Sickle Cell Trait
H	Haemophilia A	P	Thrombotic Thrombocytopenic Purpura

 

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

 

1. An 18 month old boy is brought to A&E with a grossly swollen knee after tripping over his Buzz Lightyear toy. Clinical examination and X-ray of the affected knee were normal. Hb, WBC and platelets are all normal. Clotting screen reveals a prolonged APTT but a normal PT.

 

2.  A Cypriot couple bring their two year old son to the GP having recently moved to the area. On examination the child appears pale, has hepatosplenomegaly, bossing of the skull and a skull X-ray reveals a “hair-on-end” appearance.

 

3. A seven year old girl attends A&E with a two day history of diarrhoea, vomiting and haematuria. On examination she has a fever and is hypertensive. Full blood count reveals anaemia and thrombocytopenia but the clotting screens all come back as normal. Her blood film reveals schistocytes. When you take the history her mother mentions she had been on a school trip to a local farm a few days previously.

 

4. A four year old girl is found to be anaemic. She has a normal Coombs’ test, and on examination you find her to be jaundiced with splenomegaly. Blood film reveals microspherocytes.

 

5. A six year old Afro-Caribbean boy is brought to A&E complaining of chest pain and shortness of breath. Hb is 6.2g/dL and MCV is 84fl. Haemoglobin electrophoresis revealed an absence of HbA.

 

 

ANSWERS

1. H

2. C

3. G

4. K

5. N