Diagnostic criteria for polycythemia vera

World Health Organisation, 2008

Diagnosis requires both major criteria and one minor criterion, or the first major criterion plus two minor criteria

Major criteria
  1. Hemoglobin >18.5 g/dL in men, >16.5 g/dL in women or other evidence of increased red cell volume (see below)
  2. Presence of JAK2 V617F or other functionally similar mutation (such as JAK2 exon 12 mutation)
Minor criteria
  1. Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) with prominent erythroid, granulocytic and megakaryocytic proliferation
  2. Serum erythropoietin level below the reference range for normal
  3. Endogenous erythroid colony formation in vitro
Evidence of increased red cell volume

For fulfilment of the first major criterion

  • hemoglobin or hematocrit >99th percentile of method-specific reference range for age, sex, and altitute of residence, OR
  • hemoglobin > 17g/dL in men, > 15 g/dL in women if associated with a documented and sustained increase of at least 2 g/dL from an individual’s baseline value that cannot be attributed to correction of iron deficiency, OR
  • elevated red cell mass >25% above mean normal predicted value