Medicine · Hematological Malignancies (Leukemias, Lymphoma, Myeloma, Myeloproliferative)

A 70-year-old man with polycythemia vera (JAK2 V617F positive) has a hematocrit of 58%, platelet count 850 × 10⁹/L and has had a prior thrombotic event (DVT). He is classified as high-risk. Which cytoreductive agent is first-line for high-risk PV?

  • A Ruxolitinib
  • B Anagrelide
  • C Interferon-alpha alone
  • D Hydroxyurea (hydroxycarbamide)
Correct answer: D. Hydroxyurea (hydroxycarbamide)

Explanation

Hydroxyurea is the first-line cytoreductive therapy for high-risk polycythemia vera (age >60 or prior thrombosis) per ELN guidelines, combined with phlebotomy to target hematocrit <45% and low-dose aspirin. The landmark ELN/WHO criteria and CYTO-PV trial established hematocrit <45% as the therapeutic goal. Ruxolitinib (a JAK1/2 inhibitor) is second-line for PV intolerant or resistant to hydroxyurea (RESPONSE trial). Anagrelide is used for essential thrombocythemia. Interferon-alpha (ropeginterferon) is an alternative to HU, particularly preferred in younger patients.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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