血栓性微血管病（TMA）指的是一组具有临床和组织病理特征的多种疾病。临床上，TMA的特征是微血管性溶血性贫血（MAHA），消耗性血小板减少症和器官损伤，这是由于内皮损伤和血管阻塞引起的。有几种不同的疾病状态以不同的病理生理机制表现为TMA。这些情况与重要的发病率和死亡率相关，并需要迅速的识别和治疗。血栓性血小板减少性紫癜性肾病（TTP）和溶血性尿毒症综合征（HUS）通常被认为是TMA的原发性形式，但TMA更常与其他并发症如感染、妊娠、自身免疫性疾病或恶性高血压等共存。由于疾病特异性检测的有限可用性，决定TMA的原因是一项具有诊断挑战的任务。然而，确定潜在病因是至关重要的，因为治疗策略有所不同。我们对导致TMA的疾病的认识不断发展。最新的进展已经提高了我们对推动TMA的各种不同病因机制的理解。有针对性的治疗方法的开发已经显著改善了患者的预后。在本文中，我们回顾了不同TMA引发疾病的发病机制和临床特征。我们概述了诊断和治疗的实际方法，并讨论经验性和疾病特异性治疗策略。本文受版权保护。版权所有。本文受版权保护。版权所有。

Thrombotic microangiopathy (TMA) refers to a diverse group of diseases which share clinical and histopathologic features. TMA is clinically characterized by microangiopathic hemolytic anemia (MAHA), consumptive thrombocytopenia, and organ injury which stems from endothelial damage and vascular occlusion. There are several disease states with distinct pathophysiological mechanisms that manifest as TMA. These conditions are associated with significant morbidity and mortality and require urgent recognition and treatment. Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are traditionally considered to be primary forms of TMA, but TMA more commonly occurs in association with a co-existing condition such as infection, pregnancy, autoimmune disease, or malignant hypertension, amongst others. Determining the cause of TMA is a diagnostic challenge due to limited availability of disease-specific testing. However, identifying the underlying etiology is imperative as treatment strategies differ. Our understanding of the conditions that cause TMA is evolving. Recent advances have led to improved comprehension of the varying pathogenic mechanisms that drive TMA. Development of targeted therapeutics has resulted in significant improvements in patient outcomes. In this article we review the pathogenesis and clinical features of the different TMA-causing conditions. We outline a practical approach to diagnosis and management and discuss empiric and disease-specific treatment strategies. This article is protected by copyright. All rights reserved.This article is protected by copyright. All rights reserved.