A 55-year-old man presents with plethora, splenomegaly, and elevated haemoglobin of 20 g/dL. JAK2 V617F mutation is detected. He has a prior history of DVT. His Hct is 62%. What is the most important initial treatment goal in polycythaemia vera?

• A Maintain haematocrit <45% ✓
• B Maintain haematocrit <50%
• C Achieve complete haematological response with ruxolitinib
• D Start hydroxyurea immediately without phlebotomy

Explanation

The CYTO-PV trial demonstrated that maintaining haematocrit <45% in polycythaemia vera significantly reduced the composite of cardiovascular death and thromboembolic events compared to maintaining Hct 45–50% (HR 3.91). The target Hct <45% is now the standard of care endorsed by ELN 2018. This is achieved by phlebotomy and/or cytoreductive therapy (hydroxyurea first-line for high-risk patients). Ruxolitinib (JAK1/2 inhibitor) is reserved for hydroxyurea-resistant or intolerant patients.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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