恶性浆细胞病出血与血栓形成的发病机制和处理

Pathogenesis and Management of Hemorrhage and Thrombosis in Plasma Cell Dyscrasias——Review

由治疗多发性骨髓瘤的高效免疫调节药物沙利度胺和雷利度胺(lenolidomide)诱发的静脉血栓栓塞事件(VTE)发生率高,引起了对恶性浆细胞病患者VTE的发生率和潜在的病理生理学以及血栓预防措施的关注。而在淋巴增殖性疾患患者出血并发症相对少见。典型的发生在意义未明单克隆免疫球蛋白病的获得性血管性血友病综合征(AVWS)和原发性淀粉样变相关的获得性凝血异常两者的处理正面临挑战。本文纵览恶性浆细胞病重要的止血相关并发症,并复习了可影响克隆性浆细胞病诊断和治疗处理的其他不常见的出血和血栓性事件。由于这些止血并发症大多数不常见,相关信息来自病例回顾或系列分析。

Unexpectedly high rates of venous thromboembolic events （VTE） induced by 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 the patients with lymphoproliferative disorders, acquired von Willebrand syndrome, typically occurring in the patients with monoclonal gammopathy of unknown significance, and acquired coagulopathies associated with primary amyloidosis can present with haemorrhagic complications and both are challenges to the managemert. 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 diagnosis and therapeutic management of clonal plasma cell disorders. Due to the infrequency of most these haemostasis complications, available information is typically based on retrospective cases or series analysis.