Table of contents
Polycythemia vera
Guidelines
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).
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Screening and diagnosis
Diagnostic criteria: as per ELN 2018 guidelines, use the 2016 revised WHO criteria for diagnosis of PV.
E
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
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
A
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.
B
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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.
B
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.
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Screening for acquired von Willebrand syndrome
Diagnostic procedures
Medical management
Treatment targets: as per BSH 2019 guidelines, target hematocrit of < 45% in all patients (including low-risk patients) with PV.
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Cytoreductive therapy
Antiplatelet therapy
Iron supplementation
Management of thrombosis
Management of hemorrhage
Management of pruritus
Management of cardiovascular risk factors
Therapeutic procedures
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.
B
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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.
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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