Current Evidence and Clinical Algorithms for Targeted Management of Polycythemia Vera: Applying Trial Insights to Patient Care

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Panelists discuss how polycythemia vera treatment follows a risk-stratified approach, with all patients receiving daily aspirin and phlebotomies to maintain hematocrit below 45%, while high-risk patients (aged >60 years or history of thromboembolism) additionally require cytoreductive therapy with options including hydroxyurea, pegylated interferon alfa-2a, ropeginterferon alfa-2b, or second-line ruxolitinib, with treatment modifications based on response, tolerance, and disease progression.

Summary of Polycythemia Vera Management Algorithm

Low-Risk Polycythemia Vera (PV) Management

  • Universal measures for all PV patients:
    • Low-dose aspirin (baby aspirin) daily for thromboembolic prophylaxis
    • Phlebotomy to maintain hematocrit <45%
  • Monitoring requirements:
    • New thrombosis or bleeding events
    • Development of indications for cytoreductive therapy
  • Indications to escalate to cytoreductive therapy:
    • New thrombotic event
    • Disease-related bleeding
    • Progressive symptoms
    • Progressive splenomegaly
    • Frequent phlebotomies (≥4 per year)

High-Risk PV Management

  • Definition of high-risk:
    • Aged >60 years, OR
    • Prior history of thromboembolic event, OR
    • Both factors
  • Treatment approach:
    • Daily low-dose aspirin
    • Mandatory cytoreductive therapy
    • Target hematocrit <45%

Cytoreductive Therapy Options

  • First-line options:
    • Hydroxyurea
    • Pegylated interferon alfa-2a
    • Ropeginterferon alfa-2b
  • Second-line options:
    • Ruxolitinib (FDA-approved for second-line use)
    • Alternative cytoreductive agent not used in first line
  • Indications for switching cytoreductive therapy:
    • Loss of response
    • Continued need for phlebotomies despite high-dose therapy
    • Intolerance to current agent

This treatment algorithm emphasizes the primary treatment goal of thrombosis prevention through hematocrit control and platelet inhibition, with risk-adapted cytoreductive therapy selection for high-risk patients and those with disease complications.

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