Table of contents

Polycythemia vera


Key sources

The following summarized guidelines for the evaluation and management of polycythemia vera are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2019), the European Leukemia Net (ELN 2018), and the European Society of Medical Oncology (ESMO 2015). ...
Show more

Screening and diagnosis

Diagnostic criteria: as per ELN 2018 guidelines, use the 2016 revised WHO criteria for diagnosis of PV.
WHO criteria for polycythemia vera in men
Major criteria
Hemoglobin > 16.5 g/dL or hematocrit > 49% or increased RBC mass
Bone marrow biopsy showing hypercellularity for age with trilineage growth, including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes
Presence of JAK2 V617F or JAK2 exon 12 mutation
Minor criterion
Subnormal serum EPO level
Diagnostic criteria are not met
Create free account

Classification and risk stratification

Risk of thrombosis
As per BSH 2019 guidelines:
Use age and thrombotic history to define the risk for thrombosis in patients with PV:
high risk: age ≥ 65 years and/or prior PV-associated arterial or venous thrombosis
low risk: age < 65 years and no PV-associated thrombotic history
Consider classifying some low-risk patients into higher risk in the presence of cardiovascular risk factors, elevated WBC count, extreme thrombocytosis, or hematocrit uncontrolled with venesection.

More topics in this section

  • Prognosis

Diagnostic investigations

Genetic testing
As per BSH 2019 guidelines:
Test for JAK2 V617F mutation on a peripheral blood sample with an assay sufficiently sensitive to detect a mutant allele burden as low as 1-3% in patients with persistent, significant, and unexplained erythrocytosis.
Consider obtaining deep sequencing for high-risk mutations, such as ASXL1, SRSF2, and IDH1/2, in selected patients if the presence of these mutations may influence management.

More topics in this section

  • Screening for acquired von Willebrand syndrome

Diagnostic procedures

Bone marrow biopsy: as per ELN 2018 guidelines, perform bone marrow biopsy in all patients with suspected Philadelphia chromosome-negative myeloproliferative neoplasms, except for patients with PV with hemoglobin > 18.5 g/dL in males and > 16.5 g/dL in females.

Medical management

Treatment targets: as per BSH 2019 guidelines, target hematocrit of < 45% in all patients (including low-risk patients) with PV.

More topics in this section

  • Cytoreductive therapy

  • Antiplatelet therapy

  • Iron supplementation

  • Management of thrombosis

  • Management of hemorrhage

  • Management of pruritus

  • Management of cardiovascular risk factors

Therapeutic procedures

Therapeutic phlebotomy: as per BSH 2019 guidelines, perform phlebotomy (along with cytoreduction therapy) to maintain hematocrit < 45% for secondary prevention of thrombosis.

Perioperative care

Preoperative management: as per BSH 2019 guidelines, involve a hematologist in preoperative planning to optimize RBC count control and individualize the perioperative plan.
Show 2 more

More topics in this section

  • Perioperative management

Specific circumstances

Pregnant patients: as per BSH 2019 guidelines, ensure a collaborative approach between obstetricians and hematologists to formulate an individualized plan for the pregnancy, delivery, and postpartum period based on the previous history of thrombosis, hemorrhage, and previous pregnancies.
Show 6 more

More topics in this section

  • Patients with idiopathic erythrocytosis

  • Patients with congenital erythrocytosis

  • Patients with pulmonary disease

  • Patients with post-transplant erythrocytosis

  • Patients with leukemic transformation

  • Breastfeeding patients

  • Patients on hormonal therapy