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[CME: Polycythemia vera]

Mirjam Wellauer Atencio1, Jeroen S Goede2

  • 1Akutgeriatrie, Kantonsspital Winterthur.

Praxis
|October 2, 2019
PubMed

Insights

Polycythemia vera is a myeloproliferative disease involving three cell lines, often caused by a JAK2 mutation. Treatment focuses on phlebotomy and aspirin to prevent thrombotic events.

Area of Science:

  • Hematology
  • Oncology

Background:

  • Polycythemia vera is a myeloproliferative neoplasm characterized by the overproduction of red blood cells, white blood cells, and platelets.
  • The disease is primarily associated with a mutation in the Janus kinase 2 (JAK2) gene.

Purpose of the Study:

  • To provide an overview of polycythemia vera, including its pathophysiology, clinical manifestations, risk factors, and current treatment strategies.
  • To emphasize the importance of preventing thrombotic events in patients with polycythemia vera.

Main Methods:

  • Review of current medical literature on polycythemia vera.
  • Synthesis of information regarding disease characteristics, diagnostic criteria, and therapeutic approaches.

Main Results:

  • Polycythemia vera involves the proliferation of erythroid, megakaryocytic, and granulocytic cell lines.
  • Common symptoms include pruritus and extremity pain due to increased red blood cell mass.
  • The most frequent complications are thrombotic events, with risk factors including age >60 and prior thrombosis.

Conclusions:

  • Phlebotomy and low-dose aspirin are foundational treatments for polycythemia vera, aimed at preventing thrombotic complications.
  • Cytoreductive therapy is often necessary for patients during the disease course to manage cell proliferation.

Keywords:
Chronische myeloproliferative NeoplasieErythrozytoseJAK2 mutationJAK2-MutationMyeloproliferative diseaseThromboembolien,increased red blood cellsthrombotic events

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