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Polycythemia Vera.

Jerry L Spivak1

  • 1Hematology Division, Department of Medicine, Johns Hopkins University School of Medicine, Traylor 924, 720 Rutland Avenue, Baltimore, MD, 21205, USA. jlspivak@jhmi.edu.

Current Treatment Options in Oncology
|March 9, 2018
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Summary
This summary is machine-generated.

Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by increased red blood cells. Accurate diagnosis requires measuring red cell mass and plasma volume, not just hematocrit.

Keywords:
DiagnosisJAK2 V617FManagementNatural historyPolycythemia vera

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Area of Science:

  • Hematology
  • Oncology
  • Genetics

Background:

  • Polycythemia vera (PV) is the most common myeloproliferative neoplasm (MPN), often linked to the JAK2 V1617F mutation.
  • PV presents with erythrocytosis, leukocytosis, and thrombocytosis, frequently with splenomegaly, but can manifest with isolated findings.
  • Distinguishing true erythrocytosis from pseudoerythrocytosis in PV is challenging due to concurrent plasma volume expansion.

Purpose of the Study:

  • To clarify diagnostic challenges in Polycythemia vera (PV).
  • To emphasize the importance of accurate red cell mass and plasma volume measurements for PV diagnosis.
  • To highlight current management strategies and therapeutic considerations for PV patients.

Main Methods:

  • Review of clinical presentations and diagnostic criteria for PV.
  • Analysis of hematological parameters including hematocrit, hemoglobin, red cell mass, and plasma volume.
  • Evaluation of diagnostic utility of JAK2 V617F mutation testing and bone marrow examination.

Main Results:

  • Classical PV presentation includes erythrocytosis, leukocytosis, and thrombocytosis, but isolated findings can occur.
  • Standard hematocrit measurements can be misleading in PV due to increased plasma volume.
  • JAK2 V617F mutation testing and direct red cell mass/plasma volume measurements are crucial for accurate diagnosis, especially in cases of isolated thrombocytosis.

Conclusions:

  • Accurate diagnosis of PV necessitates direct measurement of red cell mass and plasma volume, particularly when hematocrit is normal or misleading.
  • Phlebotomy is the cornerstone of PV management, aiming for sex-specific normal hematocrit levels.
  • While PV is indolent, effective therapies exist for managing symptoms and complications, avoiding treatments that increase leukemia risk.