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

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• 作者：T. Barbui^a ; ^{tbarbui@asst-pg23.it" class="auth_mail" title="E-mail the corresponding author} ; J. Thiele^b ; H. Gisslinger^c ; G. Finazzi^d ; A.M. Vannucchi^e ; A. Tefferi^f
• 关键词：Polycythemia vera ; Essential thrombocythemia ; Prefibrotiy/early primary myelofibrosis ; Overt primary myelofibrosis ; Revision ; Clinical aspects
• 刊名：Blood Reviews
• 出版年：2016
• 出版时间：November 2016
• 年：2016
• 卷：30
• 期：6
• 页码：453-459
• 全文大小：358 K

文摘

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.