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]; Shyam Patel [3]

Overview

The diagnosis of polycythemia vera is based on the World Health Organization criteria, which was initially proposed in 2008 then revised in 2016. In general, the diagnosis of polycythemia vera requires a combination of elevated hemoglobin, which include high levels of hemoglobin, presence of JAK2 V617F mutation, hypercellularity on bone marrow biopsy, low serum erythropoietin level, and endogenous erythroid colony formation in vitro.[1] THe 2016 WHO criteria more accurately reflect the disease biology of polycythemia vera.

Diagnostic Criteria

In 2008, the World Health Organization first proposed formal criteria for the diagnosis of polycythemia vera.

Category 2008 WHO Diagnostic criteria[2]

Major criteria

  • Hemoglobin > 18.5 g/dL in men, 16.5 g/dL in women, OR Hemoglobin > 17 g/dL in men, 15 g/dL in women is associated with a sustained increase of greater than or equal to 2 grams per deciliter from the baseline that cannot be attributed to correction of iron deficiency
  • Presence of JAK2 V617F, JAK2 exon 12 mutation, or other functionally similar mutation such as JAK2 exon 12 mutation

Minor criteria

  • Serum erythropoietin level below the reference range for normal
  • Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocyte, and megakaryocyte proliferation with pleomorphic, mature megakaryocytes
  • Endogenous erythroid colony growth
  • Diagnosis requires the presence of both major criteria plus 1 minor criteron, OR the first major criterion and 2 minor criteria.


The diagnosis of polycythemia vera in the current era is based on the 2016 World Health Organization criteria.[3] This criteria was revised from the criteria proposed in 2008, as the new criteria better reflect the biology of the disease. Importantly, more people will be diagnosed with polycythemia vera given that the threshold for hemoglobin elevation has been reduced for both men and women.[1]

Category 2016 WHO Diagnostic criteria

Major criteria

  • Hemoglobin > 16.5 g/dL in men, 16.0 g/dL in women OR hematocrit 49% in men, 48% in women OR increased red cell mass*
  • Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocyte, and megakaryocyte proliferation with pleomorphic, mature megakaryocytes
  • Presence of JAK2 V617F, JAK2 exon 12 mutation, or other functionally similar mutation such as JAK2 exon 12 mutation

Minor criteria

  • Serum erythropoietin level below the reference range for normal.
  • Diagnosis requires the presence of all 3 major criteria, or the first 2 major criteria plus 1 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.

References

  1. 1.0 1.1 Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA; et al. (2007). "Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel". Blood. 110 (4): 1092–7. doi:10.1182/blood-2007-04-083501. PMID 17488875.
  2. Tefferi A, Thiele J, Vardiman JW (2009). "The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos". Cancer. 115 (17): 3842–7. doi:10.1002/cncr.24440. PMID 19472396.
  3. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM; et al. (2016). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia". Blood. 127 (20): 2391–405. doi:10.1182/blood-2016-03-643544. PMID 27069254.

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