The 2016 revision of WHO classification of myeloproliferative neoplasms: Clinical and molecular advances

T. Barbui, J. Thiele, H. Gisslinger, G. Finazzi, A. M. Vannucchi, A. Tefferi

Research output: Contribution to journalReview article

42 Scopus citations

Abstract

Clinical evidence supports the need of changing the diagnostic criteria of the 2008 updated WHO classification for polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). In JAK2-mutated patients who show characteristic bone marrow (BM) morphology, clinical studies demonstrated that a hemoglobin level of 16.5 g/dL in men and 16.0 g/dl for women or a hematocrit value of 49% in men and 48% in women are the optimal cut off levels for distinguishing JAK2-mutated ET from “masked/prodromal” PV. Therefore BM morphology was upgraded to a major diagnostic criterion. Regarding ET the key issue was to improve standardization of prominent BM features enhancing differentiation between “true” ET and prefibrotic/early primary myelofibrosis (prePMF). These two entities have shown a different epidemiology and clinical outcomes. Concerning prePMF a more explicit clinical characterization of minor criteria is mandated for an improved distinction from ET and overt PMF and accurate diagnosis and outcome prediction.

Original languageEnglish (US)
Pages (from-to)453-459
Number of pages7
JournalBlood Reviews
Volume30
Issue number6
DOIs
StatePublished - Nov 1 2016

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Keywords

  • Clinical aspects
  • Essential thrombocythemia
  • Overt primary myelofibrosis
  • Polycythemia vera
  • Prefibrotiy/early primary myelofibrosis
  • Revision

ASJC Scopus subject areas

  • Hematology
  • Oncology

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