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

被引：2

作者：

Ame, Shanti ^{[1
]}

Barraco, Fiorenza ^{[2
]}

Ianotto, Jean-Christophe ^{[3
]}

Jourdan, Eric ^{[4
]}

Rey, Jerome ^{[5
]}

Viallard, Jean-Francois ^{[6
]}

Wemeau, Mathieu ^{[7
]}

Kiladjian, Jean-Jacques ^{[8
,9
]}

机构：

[1] Inst Cancerol Strasbourg Europe ICANS, Dept Haematol, Strasbourg, France

[2] Lyon Sud Hosp Ctr, Dept Haematol, Pierre Benite, France

[3] Univ Hosp Brest, Dept Clin Hematol, Brest, France

[4] Univ Hosp Nimes, Dept Clin Haematol, Nimes, France

[5] Inst Paoli Calmettes, Dept Haematol, Marseille, France

[6] Bordeaux Univ Hosp, Dept Internal Med, Bordeaux, France

[7] Hosp Ctr Roubaix, Dept Haematol, Roubaix, France

[8] Univ Paris Cite, Hop St Louis, Ctr Invest Clin, Paris, France

[9] Univ Paris Diderot, Dept Haematol, Paris, France

来源：

EJHAEM | 2023年 / 4卷 / 03期

关键词：

diagnosis; myeloproliferative neoplasms; polycythaemia vera; primary myelofibrosis; prognosis; treatment; INTERNATIONAL WORKING GROUP; STEM-CELL TRANSPLANTATION; ESSENTIAL THROMBOCYTHEMIA; MYELOID METAPLASIA; PROGNOSTIC MODEL; PREDICT SURVIVAL; RUXOLITINIB; THERAPY; UPDATE; SPLENECTOMY;

D O I：

10.1002/jha2.734

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：779 / 791

页数：13

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