Pathology EMQ template.

Name: Serena Menashe

Candidate number:

Theme: Myeloproliferative disorders

OPTION LIST

A	Chronic Idiopathic Myelofibrosis	G	Alpha-Interferon
B	Chronic Myeloid Leukaemia	H	Anagrelide
C	Essential Thrombocythaemia	I	Aspirin, venesection and cytoreductive therapy
D	Polycythaemia Vera	J	Blood transfusion and fluid replacement therapy.
E	Pseudopolycythaemia	K	Bone marrow transplant
F	Thrombotic Thrombocytopenia	L	Hydroxycarbamide

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. Mutations in the JAK2 Kinase gene are most frequently found in which myeloproliferative disorder?

2. A 30 year old pregnant woman with sustained Platelet count of around 1600x10⁹/L presents with a history of DVT and modest splenomegaly. Bone marrow analysis shows normal cellularity excepting spontaneous megakaryocyte colonies. What is your treatment of choice?

3. A 61 year old man complains of headaches and visual disturbances. Blood tests reveal raised Hb and Hct and low Erythropoietin levels. No circulating immature cells are seen although bone marrow cytology shows spontaneous erythroid colonies. What is the most likely diagnosis?

4. A 70 year old woman presents with loss of weight, fatigue and night sweats. Examination reveals signs of Budd-Chiari syndrome. Tear drop poikilocytes are found on blood film and Bone Marrow aspiration produces a dry tap. What is the most likely diagnosis?

5. A 59 year old man presents with aquagenic pruritis, splenomegaly and gout. Following thorough investigation you diagnose him with Polycythaemia Vera. What is your treatment of choice?

ANSWERS

1. D

2. G

3. D

4. A

5. I