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

A 48-year-old man with polycythaemia vera (PV) has haematocrit >65%, platelet count 900 × 10^9/L, splenomegaly, and pruritus. He has the JAK2 V617F mutation. His 10-year cardiovascular risk is high. Beyond phlebotomy and aspirin, which cytoreductive agent is NOW preferred over hydroxyurea as FIRST-LINE for high-risk PV?

  • A Ruxolitinib (JAK1/2 inhibitor)
  • B Busulfan
  • C Hydroxyurea remains preferred first-line
  • D Interferon alpha (pegylated)
Correct answer: C. Hydroxyurea remains preferred first-line

Explanation

Current ELN 2018 and ASH guidelines maintain hydroxyurea as the standard first-line cytoreductive agent for high-risk polycythaemia vera (age >60 or prior thrombosis). Pegylated interferon-alpha (ropeginterferon alfa-2b, Besremi) has been approved and is preferred in younger patients (<60 years) or when HU is intolerable/inadequate, given its potential to reduce JAK2 allele burden and possibly modify disease. Ruxolitinib is approved for HU-resistant or intolerant PV (second-line). Busulfan is rarely used due to leukaemogenic risk.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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