What are the WHO diagnostic criteria for polycythemia vera (PV)?

Updated: Aug 20, 2019
  • Author: Srikanth Nagalla, MBBS, MS, FACP; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Answer

Diagnostic criteria for PV as per the 2016 revised World Health Organization (WHO) guidelines include three major criteria and a minor criterion. Diagnosis requires the presence of either all three major criteria or the first two major criteria and the minor criterion. [5]

Major WHO criteria are as follows:

  1. Hemoglobin >16.5 g/dL in men and >16 g/dL in women, or hematocrit >49% in men and >48% in women, or red cell mass >25% above mean normal predicted value

  2. 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)

  3. Presence of JAK2V617F or JAK2 exon 12 mutation

The minor WHO criterion is as follows:

  • Serum erythropoietin level below the reference range for normal

Criterion 2 (bone marrow biopsy) may not be required in patients who have sustained absolute erythrocytosis (in men, hemoglobin/hematocrit of >18.5 g/dL/55.5% or in women, >16.5 g/dL/49.5%) if major criterion 3 and the minor criterion are present. However, bone marrow biopsy is the only way to detect initial myelofibrosis, which is present in up to 20% of patients and may predict a more rapid progression to overt myelofibrosis. [5]


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