Polycythemia vera diagnostic criteria

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]

Overview

The diagnosis of polycythemia vera is based on the world health organization criteria, which include high levels of hemoglobin, presence of JAK2617V>F, hypercellularity on bone marrow biopsy, low serum erythropoietin levels, and endogenous erythroid colony formation in vitro.

Diagnostic Criteria

The diagnosis of polycythemia vera is based on the world health organization criteria:

Category Diagnostic criteria

Major criteria

  • Hemoglobin > 18.5 g/dL in men, 16.5 g/dL in women or other evidence of increased red cell volume*
  • Presence of JAK2617V>F or other functionally similar mutation such as JAK2 exon 12 mutation

Minor criteria

  • Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) with prominent erythroid, granulocytic, and megakaryocytic proliferation
  • Serum erythropoietin level below the reference range for normal
  • Endogenous erythroid colony formation in vitro
  • Diagnosis requires the presence of both major criteria and 1 minor criterion or the presence of the first major criterion together with 2 minor criteria.
  • Hemoglobin or hematocrit greater than 99th percentile of method-specific reference range for age, sex, altitude of residence or hemoglobin greater than 17 g/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 can not be attributed to correction of iron deficiency, or elevated red cell mass greater than 25% above mean normal predicted value.

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