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获得性血管性血友病综合征,同时伴有针对凝血因子VIII凝血活性和瑞斯托霉素诱导的血小板聚集的抑制剂。

Acquired von Willebrand syndrome with inhibitors both to factor VIII clotting activity and ristocetin-induced platelet aggregation.

作者信息

Stableforth P, Tamagnini G L, Dormandy K M

出版信息

Br J Haematol. 1976 Aug;33(4):565-73. doi: 10.1111/j.1365-2141.1976.tb03575.x.

Abstract

A case of acquired von Willebrand's syndrome (vWs) is described which appeared to be due to antibodies directed against factor VIII clotting activity (FVIIIC), factor VIII-related antigen (FVIIIRAg) and von Willebrand factor. The antibodies directed against FVIIIRAg was demonstrated by the inhibitory effect of a platelet eluate on Ristocetin-induced aggregation of normal platelets. This effect was not shown by the patient's platelet-poor plasma alone, nor could it be demonstrated in platelet eluates from 13 other patients who had antibodies to FVIIIC but in whom there was no evidence of an acquired vWs.

摘要

本文描述了一例获得性血管性血友病综合征(vWs),其病因似乎是针对凝血因子 VIII 凝血活性(FVIIIC)、因子 VIII 相关抗原(FVIIIRAg)和血管性血友病因子的抗体。血小板洗脱液对瑞斯托霉素诱导的正常血小板聚集的抑制作用证实了存在针对 FVIIIRAg 的抗体。仅患者的乏血小板血浆未显示出这种作用,在另外 13 例有针对 FVIIIC 的抗体但无获得性 vWs 证据的患者的血小板洗脱液中也未证实这种作用。

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