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← Myeloproliferative Neoplasms

HEALTH PROFESSIONAL · SOURCE READING

Disease Overview for Polycythemia Vera (PV)

Source: Myeloproliferative Neoplasms Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: September 27, 2024 · Captured 2026-09-09.

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Context: Treatment of Polycythemia Vera

To establish a diagnosis of PV, the International Consensus Classification requires that the patient meet either all three major criteria or the first two major criteria with the minor criterion.[1]

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Major Criteria

Hemoglobin greater than 16.5 g/dL in men or 16.0 g/dL in women, hematocrit greater than 49% in men or 48% in women, or elevated red cell mass greater than 25% above mean normal predicted value.

Presence of a JAK2 V617F variant or a JAK2 exon 12 variant.

Bone marrow biopsy showing age-adjusted hypercellularity with trilineage proliferation (panmyelosis), including prominent erythroid, granulocytic, and increase in pleomorphic, mature megakaryocytes without atypia.

Minor Criterion

Serum erythropoietin level below reference range.

There is no staging system for this disease.

Patients have an increased risk of cardiovascular and thrombotic events [2] and leukemic transformation (blast-phase disease) or post-PV myelofibrosis.[3-5] Age older than 67 years, leukocytosis (≥15 × 109/L), a history of thrombosis, and the presence of pathogenic variants (SRSF2) are associated with a poor prognosis.[6]

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Preserved source evidence · Not medical advice