Diagnosis and management of prefibrotic myelofibrosis

on Behalf of the Associazione Italiana per la Ricerca sul Cancro Gruppo Italiano Malattie Mieloproliferative Investigators

Research output: Contribution to journalReview article

Abstract

Introduction: The 2016 WHO classification comprises two stages of primary myelofibrosis (PMF): early/prefibrotic primary myelofibrosis (pre-PMF) and overt fibrotic PMF (overt PMF). Diagnostic criteria rely on bone marrow morphology, fibrosis grade (0–1 in pre-PMF, 2–3 in overt PMF), and clinical features (leukoerythroblastosis, anemia, leucocytosis, increased lactate dehydrogenase, and palpable splenomegaly). An accurate differentiation from essential thrombocythemia (ET) is pivotal because the two entities show different clinical presentation and outcome, in terms of survival, leukemic evolution, and rates of progression to overt myelofibrosis. Areas covered: The current review provides an overview on how to diagnose and stratify patients with pre-PMF, taking into account their definite and peculiar risk of vascular event, which is often neglected, and their milder disease course, compared with overt PMF, with the aim of improving and individualizing their counseling and management. Expert commentary: Pre-PMF is a new entity characterized by a unique combination of both a thrombo-hemorrhagic risk (that brings it closer to PV and ET) and a definite risk of disease evolution (that places pre-PMF somewhat closer to the overt PMF variant).

Original languageEnglish
Pages (from-to)537-545
Number of pages9
JournalExpert Review of Hematology
Volume11
Issue number7
DOIs
Publication statusPublished - Jul 3 2018

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Keywords

  • Diagnostic criteria
  • myelofibrosis
  • prefibrotic
  • thrombocythemia
  • WHO

ASJC Scopus subject areas

  • Hematology

Cite this

on Behalf of the Associazione Italiana per la Ricerca sul Cancro Gruppo Italiano Malattie Mieloproliferative Investigators (2018). Diagnosis and management of prefibrotic myelofibrosis. Expert Review of Hematology, 11(7), 537-545. https://doi.org/10.1080/17474086.2018.1484280