Complement Therapeutics Research Group, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.
National Renal Complement Therapeutics Centre, Royal Victoria Infirmary, Newcastle upon Tyne, UK.
Int J Lab Hematol. 2022 Sep;44 Suppl 1(Suppl 1):101-113. doi: 10.1111/ijlh.13954.
Thrombotic microangiopathy (TMA) is characterized by thrombocytopenia, microangiopathic haemolytic anaemia and end organ damage. TMAs have varying underlying pathophysiology and can therefore present with an array of clinical presentations. Renal involvement is common as the kidney is particularly susceptible to the endothelial damage and microvascular occlusion. TMAs require rapid assessment, diagnosis, and commencement of appropriate treatment due to the high morbidity and mortality associated with them. Ground-breaking research into the pathogenesis of TMAs over the past 20 years has driven the successful development of targeted therapeutics revolutionizing patient outcomes. This review outlines the clinical presentations, pathogenesis, diagnostic tests and treatments for TMAs.
血栓性微血管病(TMA)的特征为血小板减少症、微血管病性溶血性贫血和靶器官损害。TMA 的潜在病理生理学机制不同,因此可表现出多种临床表现。肾脏受累很常见,因为肾脏特别容易受到内皮损伤和微血管阻塞的影响。TMA 由于与它们相关的高发病率和死亡率,需要快速评估、诊断和开始适当的治疗。过去 20 年来,对 TMA 发病机制的突破性研究推动了靶向治疗的成功发展,从而改变了患者的预后。本综述概述了 TMA 的临床表现、发病机制、诊断检测和治疗方法。