How I treat microangiopathic hemolytic anemia in patients with cancer.

How I treat microangiopathic hemolytic anemia in patients with cancer.
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DOI:
10.1182/blood.2019003810
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发表时间:
2021-03-11
期刊:
影响因子:
20.3
通讯作者:
Scully M
Scully M
中科院分区:
医学1区
文献类型:
--
作者:
Thomas MR;Scully M

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获得性溶血性贫血的诊断和治疗可能具有挑战性。在这个由Mario Cazzola编辑的How I Treat系列中,临床专家讨论了他们治疗4种不同类别获得性溶血性贫血患者的方法。微血管病理性溶血性贫血(MAHA)伴血小板减少,提示血栓性微血管病(TMA),与血栓形成有关,影响小血管或大血管。在癌症患者中,它可能与潜在的恶性肿瘤(最初的表现或进展性疾病)、其治疗或单独的偶然诊断直接相关。在表现为TMA的癌症患者中,区分偶发性血栓性血小板减少性紫癜和非典型溶血性尿毒症综合征是至关重要的,因为他们有不同的治疗策略,并且治疗的及时影响结果。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。弥漫性血管内凝血(DIC)可能是由脓毒症引起的,也可能是由癌症本身引起的。癌症治疗可能会引起TMA,要么是剂量依赖的毒性,要么是药物依赖抗体引起的特殊免疫中介反应。在肿瘤科患者中发现的许多TMA原因对血浆交换没有反应,在可行的情况下,潜在恶性肿瘤的治疗对于控制癌症-TMA或DIC驱动的疾病都是重要的。应考虑药物引起的TMA,并停止任何可能的致病因素。我们将结合临床病例讨论癌症患者MAHA的鉴别诊断和治疗,以突出处理原则。
Diagnosis and treatment of acquired hemolytic anemia can be challenging. In this How I Treat series, edited by Mario Cazzola, clinical experts discuss their approaches to the treatment of patients with 4 different classes of acquired hemolytic anemia. 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.
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