Medical Oncology Department, Hospital Clinic Barcelona, Villarroel 170, 08036, Barcelona, Spain.
Medical Oncology Department, 401 General Military Hospital of Athens, Athens, Greece.
Support Care Cancer. 2022 Oct;30(10):8599-8609. doi: 10.1007/s00520-022-06935-5. Epub 2022 May 12.
Thrombotic microangiopathy (TMA) is a syndrome that encompasses a group of disorders defined by the presence of endothelial damage leading to abnormal activation of coagulation, microangiopathic hemolytic anemia and thrombocytopenia, occlusive (micro)vascular dysfunction, and organ damage. TMA may occur in patients with malignancy as a manifestation of cancer-related coagulopathy itself or tumor-induced TMA (Ti-TMA) as a paraneoplastic uncommon manifestation of Trousseau syndrome. TMA can also be triggered by other overlapping conditions such as infections or more frequently as an adverse effect of anticancer drugs (drug-induced TMA or Di-TMA) due to direct dose-dependent toxicity or a drug-dependent antibody reaction. The clinical spectrum of TMA may vary widely from asymptomatic abnormal laboratory tests to acute severe potentially life-threatening forms due to massive microvascular occlusion. While TMA is a rare condition, its incidence may progressively increase within the context of the great development of anticancer drugs and the emerging scenarios in supportive care in cancer. The objective of the present narrative review is to provide a general perspective of the main causes, the key work-up clues that allow clinicians to diagnose and manage TMA in patients with solid tumors who develop anemia and thrombocytopenia due to frequent overlapping causes.
血栓性微血管病(TMA)是一种综合征,包含一组以内皮损伤为特征的疾病,导致异常的凝血激活、微血管性溶血性贫血和血小板减少、闭塞(微血管)功能障碍和器官损伤。TMA 可发生在恶性肿瘤患者中,表现为癌症相关凝血功能障碍本身,或肿瘤诱导的 TMA(Ti-TMA),作为特鲁索综合征的罕见副瘤表现。TMA 也可由其他重叠疾病引起,如感染,或更常见的是作为抗癌药物的不良反应(药物诱导的 TMA 或 Di-TMA),这是由于直接剂量依赖性毒性或药物依赖性抗体反应。TMA 的临床谱差异很大,从无症状的实验室检查异常到因大量微血管阻塞而导致的急性严重潜在危及生命的形式。虽然 TMA 是一种罕见的疾病,但随着抗癌药物的巨大发展和癌症支持治疗中出现的新情况,其发病率可能会逐渐增加。本叙述性综述的目的是提供一个总体视角,介绍主要原因,以及关键的检查线索,以便临床医生诊断和管理因频繁重叠原因而发生贫血和血小板减少的实体瘤患者的 TMA。