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

A 65-year-old man has polycythaemia vera (PV) with JAK2 V617F mutation, haematocrit 58%, and a history of transient ischaemic attack. According to ELN 2018 risk stratification and management guidelines, the MOST appropriate treatment is:

  • A Phlebotomy alone to haematocrit <45%
  • B Cytoreductive therapy (hydroxyurea) plus low-dose aspirin plus phlebotomy
  • C Phlebotomy plus low-dose aspirin
  • D Ruxolitinib monotherapy
Correct answer: B. Cytoreductive therapy (hydroxyurea) plus low-dose aspirin plus phlebotomy

Explanation

ELN 2018 classifies PV risk as high-risk if age >60 OR history of thrombosis (either factor). This patient is high-risk (age 65 and prior TIA). High-risk PV requires cytoreductive therapy (hydroxyurea is first-line) in addition to phlebotomy (target haematocrit <45%) and low-dose aspirin 100 mg/day. The CYTO-PV trial confirmed that maintaining haematocrit <45% reduces CV events. Ruxolitinib is reserved for hydroxyurea-intolerant/refractory patients. Aspirin alone is insufficient for high-risk PV.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.

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