A 55-year-old man is diagnosed with polycythemia vera (PV). JAK2 V617F positive. Hematocrit is 58%, spleen is palpable 5 cm below costal margin. He has a history of stroke 3 years ago. According to current ELN guidelines, what is the appropriate management?

• A Phlebotomy alone to maintain hematocrit <45%
• B Cytoreductive therapy (hydroxyurea) + aspirin + phlebotomy to Hct <45% ✓
• C Aspirin + phlebotomy to hematocrit <45%
• D Ruxolitinib as first-line cytoreductive therapy

Explanation

This patient is high-risk PV: age >60 AND prior thrombotic event (stroke). High-risk PV requires cytoreductive therapy (hydroxyurea first-line) + low-dose aspirin + phlebotomy to maintain hematocrit <45% (target per CYTO-PV trial showing 60% reduction in cardiovascular events vs. <45% Hct control). Ruxolitinib (JAK1/2 inhibitor) is second-line after hydroxyurea failure or intolerance, per ELN 2018/2021 PV guidelines. Phlebotomy alone is insufficient for high-risk disease given thrombotic risk.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.