University of Rochester, Department of Internal Medicine, United States.
University of Rochester, Department of Hematology/Oncology, United States.
Leuk Res. 2019 Oct;85:106195. doi: 10.1016/j.leukres.2019.106195. Epub 2019 Jul 29.
Patients with multiple myeloma (MM) are susceptible to developing thrombotic microangiopathies (TMAs), an etiologically diverse group of syndromes which include atypical hemolytic uremic syndrome (aHUS) and thrombotic thrombocytopenic purpura (TTP). The TMAs are characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA), and are associated with a high mortality risk and irreversible end-organ damage when treatment is delayed. In MM patients, TMAs may be triggered by specific chemotherapies, bone marrow transplantation (BMT), and progression of underlying disease. Because many characteristics of TMAs overlap with sequelae of MM and its treatments, diagnosis requires a high index of suspicion. Furthermore, our understanding of optimal treatments for these entities is rapidly evolving and clinical practice guidelines do not yet exist. Historically, consideration of a diagnosis of TMA has prompted initiation of therapeutic plasma exchange. In this review, we present an overview of the MM-related TMAs, an approach to workup and diagnosis, and argue for initial empiric MM-related TMA treatment with eculizumab rather than plasma exchange.
患有多发性骨髓瘤（MM）的患者易发生血栓性微血管病（TMA），这是一组病因多样的综合征，包括非典型溶血性尿毒症综合征（aHUS）和血栓性血小板减少性紫癜（TTP）。TMA 的特征是血小板减少和微血管性溶血性贫血（MAHA），并且如果治疗延迟，与高死亡率和不可逆的终末器官损伤相关。在 MM 患者中，TMA 可能由特定的化疗、骨髓移植（BMT）和基础疾病的进展引发。由于 TMA 的许多特征与 MM 及其治疗的后遗症重叠，因此诊断需要高度怀疑。此外，我们对这些实体的最佳治疗方法的理解正在迅速发展，临床实践指南尚未存在。历史上，考虑 TMA 的诊断会促使开始治疗性血浆置换。在这篇综述中，我们介绍了与 MM 相关的 TMA 的概述、检查和诊断方法，并主张初始经验性 MM 相关 TMA 治疗采用依库珠单抗而不是血浆置换。
