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Pathology · Haematology: essential thrombocythaemia
Sample itemA 55-year-old woman has burning pain and redness of the fingers that is relieved by cooling. Platelet count is 900 x 10^9/L, haemoglobin is normal, and BCR-ABL testing is negative. A JAK2 V617F mutation is found. What is the most appropriate drug for her symptoms?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Low-dose aspirin
Essential thrombocythaemia is a myeloproliferative neoplasm with JAK2, CALR or MPL mutations, and microvascular occlusion produces erythromelalgia. Low-dose aspirin relieves the symptoms and reduces thrombotic risk, and cytoreduction with hydroxyurea is added in high-risk patients. Extreme thrombocytosis above about 1,000 can also cause acquired von Willebrand syndrome, so aspirin needs caution when that is suspected.
Option by option
A. Low-dose aspirin (correct)
Low-dose aspirin inhibits platelet thromboxane and relieves erythromelalgia.
B. Imatinib
Imatinib treats BCR-ABL positive CML, and the test here is negative.
C. Routine plateletpheresis
Plateletpheresis is reserved for acute life-threatening thrombosis or haemorrhage and is not routine.
D. Warfarin
Warfarin is not the standard agent for primary prevention in this disease.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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