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一名急性血栓性血小板减少性紫癜患者的血管性血友病因子异常与内皮细胞紊乱

von Willebrand factor abnormalities and endothelial cell perturbation in a patient with acute thrombotic thrombocytopenic purpura.

作者信息

Moake J L, Rudy C K, Troll J H, Weinstein M J, Colannino N M, Hong S L, Koutcher J A, Melaragno A J, Manner C E

出版信息

Am J Med Sci. 1986 Jan;291(1):47-50. doi: 10.1097/00000441-198601000-00009.

DOI:10.1097/00000441-198601000-00009
PMID:3079954
Abstract

The plasma of a 63-year-old patient with an initial acute, fatal episode of thrombotic thrombocytopenic purpura (TTP) contained agglutinated platelets and a factor VIII-related von Willebrand factor (vWF) antigen level that was elevated seven-fold above normal. Unusually large vWF multimers derived from endothelial cells were detected in her plasma at the onset of the TTP episode. This is the first patient in whom vWF abnormalities indicative of in vivo endothelial cell damage or perturbation have been found during an acute episode of TTP.

摘要

一名63岁的患者首次发生急性血栓性血小板减少性紫癜(TTP)并导致死亡,其血浆中含有凝集的血小板,且VIII因子相关血管性血友病因子(vWF)抗原水平比正常高出7倍。在TTP发作开始时,在她的血浆中检测到源自内皮细胞的异常大的vWF多聚体。这是首例在TTP急性发作期间发现表明体内内皮细胞损伤或紊乱的vWF异常的患者。

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