■血栓性血小板减少性紫癜(TTP)是一种罕见但使人衰弱的血栓性微血管病,由ADAMTS13酶严重缺乏引起。这种疾病最早是在20世纪初描述的,但是这种疾病的病理生理学在过去的三十年里才被阐明。在这篇叙述性评论中,我们将总结TTP研究和发现历史上的里程碑时刻。
■我们从1924年到2023年使用以下自由文本搜索使用PubMed搜索文献:\“血栓性血小板减少性紫癜\”,\"Moschcowitz病\",和“血栓性微血管病”。我们发现了6,917篇同行评审的文章,并对这些文章进行了分类,以获取与该综述相关的相关文献。共有46篇文章被纳入审查,其余被排除在外。
■回顾了TTP研究的历史,对这里讨论的疾病的病理生理学和治疗的理解的演变中的重大事件进行采样。关于这种疾病的性质还有很多需要了解的东西,以便开发出更具体和更少有害的治疗方法。
■对导致我们当前对TTP的理解的主要发现的概述揭示了过去一个世纪中多组医生和科学家合作的结果,由于同样的合作精神,未来可能会有更多的突破。
UNASSIGNED: Thrombotic thrombocytopenic purpura (TTP) is a rare but debilitating thrombotic microangiopathy that results from severe deficiency of the enzyme ADAMTS13. The disorder was first described in the early 20th century, but the pathophysiology of the disease has only been elucidated in the past three decades. In this narrative
review, we will summarize the milestone moments in the history of TTP research and discovery.
UNASSIGNED: We searched literature using PubMed from 1924 to 2023 using the following free text searches: \"thrombotic thrombocytopenic purpura\", \"Moschcowitz disease\", and \"thrombotic microangiopathy\". We found 6,917 peer-reviewed articles and sorted through these for relevant literature pertinent to the
review. A total of 46 articles were included for
review and the remainder were excluded.
UNASSIGNED: The history of TTP research was reviewed, with a sampling of major events in the evolution of the understanding of the pathophysiology and treatment of the disease discussed here. There remains much to be learned about the nature of the disease in order to develop more specific and less harmful treatments.
UNASSIGNED: An overview of the major discoveries that have led to our current understanding of TTP reveals the results of collaboration of multiple groups of physicians and scientists through the past century, with additional breakthroughs likely to occur in the future because of that same collaborative spirit.