A 34-year-old woman is found to have JAK2 V617F mutation positive polycythaemia vera. Her haematocrit is 54%, she has splenomegaly, and a history of one episode of deep vein thrombosis. What is the most appropriate cytoreductive treatment choice in this high-risk PV patient?
- A Interferon-alpha as sole cytoreductive agent
- B Busulfan as preferred cytoreductive agent in young patients
- C Hydroxyurea as first-line cytoreductive therapy; ruxolitinib for hydroxyurea-refractory or -intolerant patients ✓
- D Phlebotomy alone without cytoreduction is sufficient for high-risk PV
Correct answer: C. Hydroxyurea as first-line cytoreductive therapy; ruxolitinib for hydroxyurea-refractory or -intolerant patients
Explanation
High-risk PV (age > 60 or prior thrombosis) requires cytoreduction in addition to phlebotomy and low-dose aspirin. Hydroxyurea is first-line cytoreduction per ELN guidelines. Ruxolitinib (JAK1/2 inhibitor) is recommended for patients who are resistant to or intolerant of hydroxyurea (RESPONSE trial). Interferon-alpha is an alternative particularly in young women of childbearing age. Phlebotomy alone is insufficient in high-risk disease.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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