Abstract：
Thrombotic microangiopathies (TMAs) represent a complex interaction of endothelial and podocyte biology, nephron physiology, complement genetics, and oncologic therapies with host immunology. The complexity of various factors, such as molecular causes, genetic expressions, and immune system mimicking, along with incomplete penetrance, make it difficult to find a straightforward solution. As a result, there may be variations in diagnosis, study, and treatment approaches, and achieving a consensus can be challenging. Here, we review the molecular biology, pharmacology, immunology, molecular genetics, and pathology of the various TMA syndromes in the setting of cancer. Controversies in etiology, nomenclature, and points requiring further clinical, translational, and bench research are discussed. Complement-mediated TMAs, chemotherapy drug-mediated TMAs, TMAs in monoclonal gammopathy, and other TMAs central to onconephrology practice are reviewed in detail. In addition, established and emerging therapies within the US Food and Drug Administration pipeline subsequently are discussed. Finally, a comprehensive review of critical areas of onconephrology clinical practice is presented as practical value to the clinical practitioner and seeds of investigation to be sown among the community of atypical hemolytic uremic syndrome researchers.
摘要：
血栓性微血管病（TMA）代表了内皮和足细胞生物学的复杂相互作用，肾单位生理学，补充遗传学，和肿瘤治疗与宿主免疫学。各种因素的复杂性，如分子原因，基因表达，和免疫系统模仿，还有不完整的外显率，很难找到一个简单的解决方案。因此,诊断可能会有所不同，study,和治疗方法，达成共识可能具有挑战性。这里,我们回顾了分子生物学，药理学,免疫学,分子遗传学,以及癌症背景下各种TMA综合征的病理学。在病因学上有争议，命名法，以及需要进一步临床治疗的点，翻译,并讨论了实验室研究。补体介导的TMA，化疗药物介导的TMA，单克隆丙种球蛋白病中的TMAs，和其他TMA中心的健康实践进行了详细的审查。此外,随后讨论了美国食品和药物管理局管道内已建立和新兴的疗法。最后,对临床医师和非典型溶血性尿毒综合征研究人员的研究种子进行了全面的回顾，对临床医师和研究种子具有实用价值。
