Tefferi et al listed revised criteria for the diagnosis of polycythemia vera (PV). These incorporate new developments associated with the JAK2 gene. The authors are from an ad hoc international expert panel for the World Health Organization (WHO).


Major criteria:

(1) evidence of increased red cell volume - one or more of the following:

(1a) hemoglobin > 18.5 g/dL in a male

(1b) hemoglobin > 16.5 g/dL in a female

(1c) hemoglobin or hematocrit > 99% for age, gender, race and altitude of residence

(1d) elevated hemoglobin (> 17 g/dL for male, > 15 g/dL for female AND (hemoglobin >= 2 g/dL above baseline) AND (elevation sustained) AND (not a rebound due to correction of iron deficiency or other anemia)

(1e) red cell mass > 125% above the mean predicted value

(2) presence of JAK2 mutation (JAK2 617V>F, JAK2 exon 12 mutation, other functionally similar)


Minor criteria:

(1) hypercellular bone marrow for age with trilineage proliferation (panmyelosis involving erythroid, granulocytic and megakaryocytic precursors)

(2) serum erythropoietin concentration below the lower limit for the normal reference range

(3) in vitro formation of endogenous erythroid colonies

Number of Major Criteria

Number of Minor Criteria

Diagnosis of PV


>= 1


first major

>= 2



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