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Polycythemia vera: past, present and future.

Ami B Patel1, Lucia Masarova2, Ruben A Mesa3

  • 1Division of Hematology and Hematologic Malignancies, The University of Utah, Salt Lake City, UT, USA.

Leukemia & Lymphoma
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PubMed
Summary

Novel therapies for polycythemia vera (PV) now focus on modifying the disease by targeting JAK2-mutant cells. Ropeinterferon and JAK inhibitors show disease-modifying effects, transforming PV treatment paradigms.

Keywords:
erythrocytosismyeloproliferative disorders < neoplasiapolycythemiapolycythemia verasignaling therapies < neoplasia

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Area of Science:

  • Hematology
  • Oncology
  • Pharmacology

Background:

  • Polycythemia vera (PV) historically focused on symptom and risk management.
  • Current research emphasizes disease modification by targeting JAK2-mutant stem/progenitor cells.

Purpose of the Study:

  • To review past, current, and future therapeutic strategies for PV.
  • To highlight key clinical studies and their impact on treatment paradigms.

Main Methods:

  • Review of clinical studies on novel PV therapies.
  • Analysis of data from LOW-PV, CONTINUATION-PV, and MAJIC-PV trials.

Main Results:

  • Ropeinterferon demonstrates a disease-modifying effect in PV.
  • JAK inhibitors also show potential for disease modification in PV.
  • Novel agents like hepcidin mimetics aim for phlebotomy-free management.

Conclusions:

  • The treatment paradigm for PV is shifting towards disease modification.
  • Prospective molecular monitoring is crucial for future PV clinical trials.
  • Interferon therapy and JAK inhibitors represent significant advancements in PV management.