Pathology EMQ template.

Name: Nikila Patil

Theme: Paediatric Haematology

OPTION LIST

A	Acute Lymphoblastic Leukaemia	I	Henoch-Schonlein Purpura
B	Beta-Thalassaemia	J	Hereditary Spherocytosis
C	Diamond-Blackfan Syndrome	K	Hodgkin’s Lymphoma
D	Dyskeratosis Congenita	L	Rhesus Haemolytic Disease
E	Fanconi Anaemia	M	Shwachman-Diamond Syndrome
F	G6PD Deficiency	N	Sickle Cell Anaemia
G	Haemophilia A	O	Thrombocytopenia-Absent Radius Syndrome
H	Haemophilia B	P	Von Willebrand Disease

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 neonate develops jaundice within a few hours of birth. A blood smear reveals Howell-Jolly bodies. Osmotic fragility test is positive.

2. A neonate is found to have a cleft palate and abnormal thumbs. Investigations reveal a macrocytic anaemia.

3. A four year old boy comes in with generalised fatigue, bruising and a history of frequent infections. On examination the boy has palpable neck swellings.

4. A 3 month old infant with absent radii bilaterally but normal thumbs, presents with bruising, petechiae and nosebleeds.

5. A 2 year old boy presents with bleeding gums. On further investigation Hb, WBC and platelets are normal. APTT is prolonged, bleeding time is prolonged and PT is normal.

ANSWERS

1. J

2. C

3. A

4. O

5. P