A 48-year-old man is found to have JAK2 V617F mutation positive polycythemia vera. Hematocrit is 56%, platelet count 680 × 10⁹/L. He is a heavy smoker with one prior TIA. According to current ELN high-risk PV management guidelines, the appropriate treatment is:

• A Phlebotomy alone targeting Hct <45%
• B Phlebotomy plus low-dose aspirin only
• C Phlebotomy + low-dose aspirin + cytoreductive therapy with hydroxyurea ✓
• D Ruxolitinib as first-line cytoreduction

Explanation

This patient is high-risk PV (age >60 OR prior thrombosis — he has prior TIA). High-risk PV requires cytoreductive therapy in addition to phlebotomy and aspirin. Hydroxyurea is the first-line cytoreductive agent per ELN 2021 guidelines. The CYTO-PV trial confirmed targeting hematocrit <45% reduces thrombosis. Phlebotomy alone is insufficient for high-risk PV. Ruxolitinib is second-line, used when HU fails or causes intolerance. Low-dose aspirin (81–100 mg/day) reduces thrombotic risk in all PV patients without contraindications.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.