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浆细胞异常增殖性疾病中出血与血栓形成的发病机制及管理

Pathogenesis and management of bleeding and thrombosis in plasma cell dyscrasias.

作者信息

Eby Charles

机构信息

Department of Pathology & Immunology and Medicine, Washington University School of Medicine, St Louis, MO 63110, USA.

出版信息

Br J Haematol. 2009 Apr;145(2):151-63. doi: 10.1111/j.1365-2141.2008.07577.x. Epub 2009 Feb 4.

Abstract

Unexpectedly high rates of venous thromboembolic events (VTE) concurrent with the introduction of highly effective immune modulating drugs thalidomide and lenolidomide for treatment of multiple myeloma have focused attention on the incidence and underlying pathophysiology of VTE in patients with plasma cell dyscrasias, and on thromboprophylaxis approaches. While bleeding complications are relatively uncommon in patients with lymphoproliferative disorders, acquired von Willebrand syndrome, typically occurring in patients with monoclonal gammopathy of unknown significance, and acquired coagulopathies associated with primary amyloidosis can present with haemorrhagic complications and both are challenging to manage. This review highlights these important haemostasis-related complications of plasma cell dyscrasias and provides an overview of other uncommon bleeding and thrombotic events that can affect diagnostic and therapeutic management of clonal plasma cell disorders. Due to the infrequency of most of these haemostasis complications, available information is typically based on retrospective cases or series.

摘要

在使用高效免疫调节药物沙利度胺和来那度胺治疗多发性骨髓瘤时,静脉血栓栓塞事件(VTE)的发生率意外升高,这使得人们关注浆细胞发育异常患者VTE的发生率及潜在病理生理学,以及血栓预防方法。虽然出血并发症在淋巴增殖性疾病患者中相对不常见,但获得性血管性血友病综合征(通常发生在意义未明的单克隆丙种球蛋白病患者中)以及与原发性淀粉样变性相关的获得性凝血病可出现出血并发症,且二者的治疗都具有挑战性。本综述重点介绍了浆细胞发育异常这些重要的止血相关并发症,并概述了其他可能影响克隆性浆细胞疾病诊断和治疗管理的罕见出血和血栓形成事件。由于这些止血并发症大多不常见,现有信息通常基于回顾性病例或系列研究。

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