A patient with polycythaemia vera (PV) has a haematocrit of 62%, splenomegaly, and pruritus. JAK2 V617F mutation is confirmed. His cardiovascular risk is high (age 65, prior DVT). Which management is most appropriate?

• A Phlebotomy alone to haematocrit < 45%
• B Phlebotomy to maintain haematocrit < 45% plus cytoreduction with hydroxyurea plus low-dose aspirin ✓
• C Ruxolitinib as first-line cytoreductive therapy
• D Phlebotomy to haematocrit < 50% with observation only

Explanation

In high-risk PV (age ≥ 60 or prior thrombosis), the CYTO-PV trial confirmed the haematocrit target < 45% reduces thrombosis risk significantly versus the < 50% target. Cytoreduction with hydroxyurea is indicated for high-risk patients alongside phlebotomy and low-dose aspirin. Ruxolitinib is reserved for hydroxyurea failure or intolerance (RESPONSE trial). Phlebotomy alone is inadequate for high-risk PV due to persistent thrombotic risk from residual myeloproliferation. The correct combination is all three — phlebotomy, hydroxyurea, and aspirin.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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