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 language | English (US) |
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Pages (from-to) | 453-459 |
Number of pages | 7 |
Journal | Blood Reviews |
Volume | 30 |
Issue number | 6 |
DOIs | |
State | Published - Nov 1 2016 |
Keywords
- Clinical aspects
- Essential thrombocythemia
- Overt primary myelofibrosis
- Polycythemia vera
- Prefibrotiy/early primary myelofibrosis
- Revision
ASJC Scopus subject areas
- Hematology
- Oncology