WHO diagnostic criteria polycythemia vera

Major criteria

Hemoglobin >16.5 g/dL in men

Hemoglobin >16.0 g/dL in women

Hematocrit >49% in men

Hematocrit >48% in women

Increased red cell mass*

Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size)

Presence of JAK2 V617F or JAK2 exon 12 mutation

Minor criterion

Subnormal serum erythropoietin level

Diagnosis of PV requires meeting either all 3 major criteria, or the first 2 major criteria and the minor criterion^¶

MF: myelofibrosis; PV: polycythemia vera; WHO: World Health Organization
* More than 25% above mean normal predicted value.
¶ Criterion number 2 (bone marrow biopsy) may not be required in cases with sustained absolute erythrocytosis: hemoglobin levels >18.5 g/dL in men (hematocrit, 55.5%) or >16.5 g/dL in women (hematocrit, 49.5%) if major criterion 3 and the minor criterion are present. However, initial myelofibrosis (present in up to 20% of patients) can only be detected by performing a bone marrow biopsy; this finding may predict a more rapid progression to overt myelofibrosis (post-PV MF).

Republished with permission of the American Society of Hematology, from Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016; 127:2391. Copyright © 2016; permission conveyed through Copyright Clearance Center, Inc.

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WHO diagnostic criteria polycythemia vera