Liu J, Hutzler M, Li C, Pechet L
Department of Pathology, University of Massachusetts Memorial Health Care, Worcester, MA 01605, USA.
J Thromb Thrombolysis. 2001 May;11(3):261-72. doi: 10.1023/a:1011921122595.
TTP and HUS are two disorders with many similarities. Though their first descriptions appeared at different time in history, there has been a trend among physicians to consider them as the same clinical entity. However, in recent years new research findings on the pathophysiology of TTP and HUS have revealed some differences between the two disorders. In this paper, we will review the current approaches to the clinical and laboratory diagnosis of TTP and HUS, as well as therapeutic strategies. We will also summarize the recent advances in three areas in the study of the pathophysiology of TTP and HUS, namely the newly discovered von Willebrand factor multimer-cleaving protease, endothelial cell apoptosis induced by serum from patients with TTP and atypical HUS and the activation of complement system. Since distinguishing and differentiating between TTP and HUS may help to develop more effective therapies targeted at key steps of the disease development, we will discuss possible ways of reclassifying the TTP-HUS disorders. In the end, we also present our views on possible future development.
血栓性血小板减少性紫癜（TTP）和溶血尿毒综合征（HUS）是两种有许多相似之处的疾病。尽管它们最早的描述出现在历史上的不同时期，但医生们一直倾向于将它们视为同一临床实体。然而，近年来关于TTP和HUS病理生理学的新研究发现揭示了这两种疾病之间的一些差异。在本文中，我们将综述TTP和HUS临床及实验室诊断的当前方法以及治疗策略。我们还将总结TTP和HUS病理生理学研究三个领域的最新进展，即新发现的血管性血友病因子多聚体裂解蛋白酶、TTP和非典型HUS患者血清诱导的内皮细胞凋亡以及补体系统的激活。由于区分TTP和HUS可能有助于开发针对疾病发展关键步骤的更有效疗法，我们将讨论重新分类TTP-HUS疾病的可能方法。最后，我们还对未来可能的发展提出了看法。
