Pathology EMQ template.

Name: Sanjay Mehta

Theme: Paediatric Haematology

 

OPTION LIST

 

A	Acute Myeloid Leukaemia	I	Haemoglobin Barts Hydrops Fetails
B	Alloimmune haemolytic anaemia	J	Haemolytic uraemic syndrome
C	Autoimmune Thrombocytopenia	K	Hereditary Elliptocytosis
D	Beta thalassaemia intermediate	L	Hereditary Spherocytosis
E	Beta thalassaemia major	M	Sickle cell trait
F	Chronic Myeloid Leukaemia	N	Pyruvate Kinase deficiency
G	Diamond-Blackfan Anaemia	O	Thrombotic Thrombocytopenic Purpura
H	Glucose-6-phosphate dehydrogenase (G6PD) deficiency	P	Transient Abnormal Myelopoiesis

 

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 3 year old male presents to his GP with jaundice and splenomegaly. A blood film reveals the presence of microspherocytes and reticulocytes. Both serum bilirubin and urinary urobiligen are raised. The Coombs’ test returns with a negative result.

 

2. A mother of Greek origin attends A&E, because she is worried about her 2 month old son, who is crying and has developed sudden pallor and jaundice. Irregularly contracted cells and polychromatic macrocytes are seen on the blood film, and mild splenaomegaly is felt during the examination. The mother also says that he has a 2 day history of fever and has noticed that his urine has been very dark when she has been changing his nappy. 

 

3. A 5 year old girl is brought to his GP by her parents, who have recently moved to the area and are worried about their daughter’s pale appearance. They also complain that she looks small compared to their neighbour’s 5 year old daughter. On examination, she has enlarged maxilla and prominent frontal and parietal bones, as well as hepatosplenomegaly. A skull X-ray reveals expansion of the medullary cavity giving rise to a ‘hair on end’ appearance.

 

4. A 3 year old boy presents to A&E following a viral illness with purpuric rash, epistaxis and ecchymoses. The FBC reveals a normal Hb and WCC, with a platelet count of 17x10⁹/L. Bone marrow show increased numbers of megakaryocytes.

 

5. On routine blood testing, a neonate diagnosed with Down’s Syndrome is noted to have increased numbers of circulating megakaryocyte blast cells and nucleated red blood cells. However, a blood film is requested 2 months later and returns as ‘normal’.

 

ANSWERS

1. G

2. E

3. H

4. A

5. P