Medical College of Wisconsin, Department of Pathology, Milwaukee, WI 53226.
The University of Chicago, Department of Pathology, Chicago, IL 60637.
Semin Diagn Pathol. 2020 May;37(3):121-126. doi: 10.1053/j.semdp.2020.01.002. Epub 2020 Feb 6.
Thrombotic microangiopathy (TMA) is characterized by thrombocytopenia and microangiopathic hemolytic anemia, results from acute and/or chronic endothelial cell injury, and often manifests with kidney dysfunction. TMA can be observed in a wide spectrum of clinical scenarios, which includes but is not limited to thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, severe (malignant) hypertension, preeclampsia/eclampsia, antiphospholipid antibody syndrome, scleroderma renal crisis, drug toxicities, or metabolic disorders. These different conditions are impossible to distinguish based solely on the pathologic findings, necessitating correlation with clinical and laboratory data. For both treating physicians and pathologists, the absence of specific pathologic features for a particular etiology or association with TMA remains a great source of frustration and confusion that currently accompanies this complex topic. In this review, we introduce a new paradigm for TMA that coalesces around the important contribution of the complement system, which has potential implications for therapeutic management, disease recurrence in the kidney allograft, and genetic risks to family members.
血栓性微血管病（TMA）的特征是血小板减少症和微血管性溶血性贫血，由急性和/或慢性内皮细胞损伤引起，常表现为肾功能障碍。TMA 可在广泛的临床情况下观察到，包括但不限于血栓性血小板减少性紫癜、溶血尿毒综合征、严重（恶性）高血压、子痫/子痫、抗磷脂抗体综合征、硬皮病肾危象、药物毒性或代谢紊乱。这些不同的情况仅根据病理发现无法区分，需要与临床和实验室数据相关联。对于治疗医生和病理学家来说，缺乏特定病因的特定病理特征或与 TMA 相关联仍然是一个令人沮丧和困惑的主要来源，这与这个复杂的主题有关。在这篇综述中，我们介绍了一个围绕补体系统重要贡献的 TMA 新范式，这对治疗管理、肾脏移植物中的疾病复发以及家庭成员的遗传风险具有潜在意义。
