Polycythemia Vera
The World Health Organization (WHO) classification of myeloproliferative neoplasms (MPN) [1,2] was revised in 2008 and defines the diagnosis of Polycythemia vera (PV) according to the following algorithm:
A patient must meet either:
- Both major criteria (Nr. 1 and Nr. 2) and one minor criterion
- Major Criterion Nr. 1 and two minor criteria
Major Criteria:
- The patient must have one of the following:
- Hgb > 18.5 g/dL (men) or Hgb > 16.5 g/dL (women)
- Hgb > 17 g/dL (men) or Hgb > 15 g/dL if associate with a sustained increase of > 2 g/dL from baseline that cannot be attributed to a correction of iron deficiency.
- Hgb or hematocrit greater than the 99th percentile of reference range for age, sex, or altitude of residence.
- Red cell mass >25% above the mean normal predicted.
- Presence of the JAK2 V617F mutation or a similar mutation.
Minor Criteria:
- Bone marrow trilineage myeloproliferation
- Subnormal serum Erythropoietin (Epo) levels
- Growth of Endogeneous Erythroid Colonies (EECs)
References
- Swerdlow S, Campo E, Harris N. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IRAC Press. 2008:Lyon, France.
- Tefferi A, Thiele J, Vardiman JW. The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. Cancer. 2009;115(17):3842-3847.
- Visit also the FIM (French Intergroup MPD) website.
- Hussein et al. Clinical utility gene card for: familial polycythaemia vera European Journal of Human Genetics 2012, e1–e4; doi:10.1038/ejhg.2012.216