A 58-year-old man is found to have Hb 19.8 g/dL, PCV 61%, WBC 13,500/µL, and spleen +6 cm below costal margin. JAK2 V617F mutation is positive. He has a history of a deep vein thrombosis 2 years prior. According to revised WHO/ELN criteria for polycythaemia vera, what is the MOST appropriate treatment?
- A Cytoreductive therapy with hydroxycarbamide (hydroxyurea) plus aspirin plus phlebotomy ✓
- B Phlebotomy alone to target haematocrit <45%
- C Phlebotomy to target haematocrit <45% plus low-dose aspirin
- D Ruxolitinib (JAK inhibitor) as first-line cytoreductive therapy
Correct answer: A. Cytoreductive therapy with hydroxycarbamide (hydroxyurea) plus aspirin plus phlebotomy
Explanation
This patient has high-risk PV by ELN criteria: age >60 years OR prior thrombotic event. High-risk PV requires cytoreductive therapy to reduce thrombotic risk, in addition to phlebotomy to target PCV <45% (the CYTOPV trial proved PCV <45% reduces CV events) and low-dose aspirin. Hydroxycarbamide (hydroxyurea) is first-line cytoreductive therapy. Phlebotomy alone is appropriate only for low-risk PV (age <60, no thrombosis, no extreme cytokinesis). Ruxolitinib is second-line for hydroxycarbamide-refractory/intolerant PV (RESPONSE trial), not first-line.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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