Division of Hematology and Medical Oncology, Knight Cancer Institute, Oregon Health & Science University, 3181 Southwest Sam Jackson Park Road, Portland, OR 97239, USA.
Division of Hematology and Medical Oncology, The Hemophilia Center, Portland VA Medical Center, Knight Cancer Institute, Oregon Health & Science University, 3181 Southwest Sam Jackson Park Road, L586, Portland, OR 97239, USA.
Med Clin North Am. 2017 Mar;101(2):395-415. doi: 10.1016/j.mcna.2016.09.010. Epub 2016 Dec 27.
Thrombotic thrombocytopenia purpura (TTP) and the hemolytic uremic syndrome (HUS) are rare thrombotic microangiopathies that can be rapidly fatal. Although the acquired versions of TTP and HUS are generally highest on this broad differential, multiple rarer entities can produce a clinical picture similar to TTP/HUS, including microangiopathic hemolysis, renal failure, and neurologic compromise. More recent analysis has discovered a host of genetic factors that can produce microangiopathic hemolytic syndromes. This article discusses the current understanding of thrombotic microangiopathy and outlines the pathophysiology and causative agents associated with each distinct syndrome as well as the most accepted treatments.
血栓性血小板减少性紫癜（TTP）和溶血尿毒综合征（HUS）是罕见的血栓性微血管病，可迅速致命。虽然获得性 TTP 和 HUS 通常是该广泛鉴别诊断中最常见的，但许多更罕见的疾病也可产生类似于 TTP/HUS 的临床表现，包括微血管性溶血性贫血、肾衰竭和神经功能障碍。最近的分析发现了一系列遗传因素，这些因素可导致微血管性溶血性综合征。本文讨论了对血栓性微血管病的现有认识，并概述了与每种独特综合征相关的病理生理学和病因以及最被接受的治疗方法。
