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我如何治疗癌症患者的微血管病性溶血性贫血。

How I treat microangiopathic hemolytic anemia in patients with cancer.

机构信息

Department of Haematology, University College London Hospital, Cardiometabolic Programme-NIHR UCLH/UCL BRC, London, United Kingdom.

出版信息

Blood. 2021 Mar 11;137(10):1310-1317. doi: 10.1182/blood.2019003810.

Abstract

Microangiopathic hemolytic anemia (MAHA) with thrombocytopenia, suggests a thrombotic microangiopathy (TMA), linked with thrombus formation affecting small or larger vessels. In cancer patients, it may be directly related to the underlying malignancy (initial presentation or progressive disease), to its treatment, or a separate incidental diagnosis. It is vital to differentiate incidental thrombotic thrombocytopenia purpura or atypical hemolytic uremic syndrome in cancer patients presenting with a TMA, as they have different treatment strategies, and prompt initiation of treatment impacts outcome. In the oncology patient, widespread microvascular metastases or extensive bone marrow involvement can cause MAHA and thrombocytopenia. A disseminated intravascular coagulation (DIC) picture may be precipitated by sepsis or driven by the cancer itself. Cancer therapies may cause a TMA, either dose-dependent toxicity, or an idiosyncratic immune-mediated reaction due to drug-dependent antibodies. Many causes of TMA seen in the oncology patient do not respond to plasma exchange and, where feasible, treatment of the underlying malignancy is important in controlling both cancer-TMA or DIC driven disease. Drug-induced TMA should be considered and any putative causal agent stopped. We will discuss the differential diagnosis and treatment of MAHA in patients with cancer using clinical cases to highlight management principles.

摘要

微血管性溶血性贫血(MAHA)伴血小板减少症提示血栓性微血管病(TMA),与影响小血管或大血管的血栓形成有关。在癌症患者中,它可能与潜在的恶性肿瘤(初始表现或进行性疾病)、其治疗或单独的偶发诊断直接相关。区分癌症患者 TMA 中偶然发生的血栓性血小板减少性紫癜或非典型溶血尿毒综合征非常重要,因为它们有不同的治疗策略,及时开始治疗会影响预后。在肿瘤患者中,广泛的微血管转移或广泛的骨髓受累可导致 MAHA 和血小板减少症。败血症可引发弥散性血管内凝血(DIC),或由癌症本身驱动。癌症治疗可能会导致 TMA,要么是剂量依赖性毒性,要么是由于药物依赖性抗体引起的特发性免疫介导反应。肿瘤患者中许多 TMA 的病因对血浆置换无反应,在可行的情况下,治疗潜在的恶性肿瘤对于控制癌症-TMA 或 DIC 驱动的疾病非常重要。应考虑药物诱导的 TMA,并停止任何可能的致病药物。我们将使用临床病例讨论癌症患者 MAHA 的鉴别诊断和治疗,以突出管理原则。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/84da/8555418/29bb8b21f31f/bloodBLD2019003810Cabsf1.jpg

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