血栓性血小板减少性紫癜研究史:叙事性回顾。

Annals of blood Pub Date : 2024-06-30 Epub Date: 2024-02-16 DOI:10.21037/aob-23-46
Konstantine Halkidis, Bernhard Lämmle, X Long Zheng
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引用次数: 0

摘要

背景和目的:血栓性血小板减少性紫癜(TTP)是一种罕见但却使人衰弱的血栓性微血管病,是由于严重缺乏 ADAMTS13 酶所致。这种疾病在 20 世纪初首次被描述,但其病理生理学直到过去 30 年才被阐明。在这篇叙述性综述中,我们将总结 TTP 研究和发现史上具有里程碑意义的时刻:我们使用 PubMed 检索了 1924 年至 2023 年的文献,并使用以下自由文本检索:"血栓性血小板减少性紫癜"、"Moschcowitz 病 "和 "血栓性微血管病"。我们找到了 6917 篇经同行评审的文章,并对这些文章进行了分类,以找到与本综述相关的文献。共有 46 篇文章被纳入综述,其余文章被排除在外:我们回顾了TTP的研究历史,并在此讨论了对该疾病的病理生理学认识和治疗演变过程中的一些重大事件。为了开发更具针对性、危害更小的治疗方法,我们仍需对该疾病的本质进行深入了解:综观我们目前对 TTP 的认识所取得的重大发现,可以看出多个医生和科学家团体在过去一个世纪中通力合作的成果,而这种合作精神也将在未来带来更多突破。
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The history of thrombotic thrombocytopenic purpura research: a narrative review.

Background and objective: 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.

Methods: 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.

Key content and findings: 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.

Conclusions: 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.

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