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Review
. 2023 Jun 7;4(3):779-791.
doi: 10.1002/jha2.734. eCollection 2023 Aug.

Advances in management of primary myelofibrosis and polycythaemia vera: Implications in clinical practice

Shanti Amé  1 , Fiorenza Barraco  2 , Jean-Christophe Ianotto  3 , Eric Jourdan  4 , Jérôme Rey  5 , Jean-François Viallard  6 , Mathieu Wémeau  7 , Jean-Jacques Kiladjian  8
Affiliations
Review

Advances in management of primary myelofibrosis and polycythaemia vera: Implications in clinical practice

Shanti Amé et al. EJHaem. .

Abstract

Primary myelofibrosis (PMF) and polycythaemia vera (PV) are rare BCR-ABL1-negative myeloproliferative neoplasms, associated with an increased risk of thrombosis, haemorrhagic complications and progression to fibrosis or leukaemia or fibrosis for PV. Both diseases are characterised by biological and clinical heterogeneity, leading to great variability in their management in routine clinical practice. In this review, we present an updated overview of the diagnosis, prognosis and treatment of PMF and PV, and we discuss how our multidisciplinary expert group based across France translates this evidence-based knowledge into routine clinical practice.

Keywords: diagnosis; myeloproliferative neoplasms; polycythaemia vera; primary myelofibrosis; prognosis; treatment.

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Conflict of interest statement

Shanti Amé participated in advisory boards for Bristol Myers Squibb and Novartis. Fiorenza Barraco declares no competing financial interests. Jean‐Christophe Ianotto received speaker fees from Novartis. Eric Jourdan participated in advisory boards for Bristol Myers Squibb, Novartis and AbbVie. Jérôme Rey participated in advisory boards for Bristol Myers Squibb, Novartis and AbbVie. Jean‐François Viallard received speaker fees from AOP Orphan, Novartis, Bristol Myers Squibb, AbbVie and Gilead. Mathieu Wémeau participated in advisory boards for AbbVie, AOP Orphan, Bristol Myers Squibb and Novartis, and received speaker fees from Novartis. Jean‐Jacques Kiladjian received consulting fees from AbbVie and Novartis, speaker fees from AOP Orphan, Novartis and Bristol Myers Squibb, and participated in advisory boards for Incyte.

Figures

FIGURE 1
FIGURE 1
Treatment algorithm for patients with primary myelofibrosis (PMF). HR, high‐risk; JAKi, Janus kinase inhibitors; MIPSS70, Mutation‐Enhanced International Prognostic Score System for Transplantation‐Age Patients; VHR, very high risk.
FIGURE 2
FIGURE 2
Risk‐stratified treatment algorithm of polycythaemia vera (PV). Hct, haematocrit.
See this image and copyright information in PMC

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