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原发性骨髓纤维化和真性红细胞增多症的管理进展:对临床实践的启示

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

作者信息

Amé Shanti, Barraco Fiorenza, Ianotto Jean-Christophe, Jourdan Eric, Rey Jérôme, Viallard Jean-François, Wémeau Mathieu, Kiladjian Jean-Jacques

机构信息

Department of Haematology Institut de Cancérologie Strasbourg Europe (ICANS) Strasbourg France.

Department of Haematology Lyon Sud Hospital Centre Pierre-Bénite France.

出版信息

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

Abstract

Primary myelofibrosis (PMF) and polycythaemia vera (PV) are rare -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.

摘要

原发性骨髓纤维化(PMF)和真性红细胞增多症(PV)是罕见的阴性骨髓增殖性肿瘤,与血栓形成、出血并发症以及PV进展为纤维化、白血病或纤维化的风险增加相关。这两种疾病均具有生物学和临床异质性,导致其在常规临床实践中的管理存在很大差异。在本综述中,我们对PMF和PV的诊断、预后及治疗进行了最新概述,并讨论了我们遍布法国的多学科专家组如何将这一基于证据的知识转化为常规临床实践。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/99c5/10435696/9b0d5eab5c03/JHA2-4-779-g002.jpg

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