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不同类型血管性血友病的血浆制品治疗

Plasma product treatment in various types of von Willebrand's disease.

作者信息

Berntorp E

机构信息

Department of Coagulation Disorders, University of Lund, Malmö General Hospital, Sweden.

出版信息

Haemostasis. 1994 Sep-Oct;24(5):289-97. doi: 10.1159/000217116.

Abstract

Four different virus-inactivated factor VIII concentrates (Haemate P, Behring; Profilate, Alpha, FVIII-VHP-vWF, CRTS), near-pure von Willebrand factor (Facteur Willebrand, CRTS) or one recombinant FVIII preparation (Recombinate, Baxter) were given to one or more patients with different forms of von Willebrand's disease. Duke bleeding time, VIII:C, vWF:Ag, RC of activity, and the multimeric pattern of plasma vWF were monitored. Both Duke bleeding time and the multimeric pattern were normalized after treatment with Haemate P, FVIII-VHP-vWF, or Facteur Willebrand, and to a lesser extent after Profilate. Except in one case, the reduction in bleeding time lasted longer after Haemate P than after the other concentrates. Recombinate had no effect on primary hemostasis, and the half-life of VIII:C was very short. If prompt hemostasis is required, and when pharmacological correction of the defect is impossible, we recommend a concentrate containing both FVIII and the full complement of vWF multimers, but for prophylactic treatment pure von Willebrand factor may be used.

摘要

四种不同的病毒灭活因子VIII浓缩物(海莫莱士,拜耳公司生产;普洛法特,阿尔法公司生产,FVIII-VHP-vWF,CRTS公司生产)、近纯的血管性血友病因子(血管性血友病因子,CRTS公司生产)或一种重组因子VIII制剂(瑞友宁,百特公司生产)被给予了一名或多名患有不同类型血管性血友病的患者。监测了杜克出血时间、VIII:C、vWF:Ag、活性回收率以及血浆vWF的多聚体模式。在用海莫莱士、FVIII-VHP-vWF或血管性血友病因子治疗后,杜克出血时间和多聚体模式均恢复正常,在用普洛法特治疗后恢复程度较小。除了1例患者外,海莫莱士治疗后出血时间的缩短持续时间比其他浓缩物更长。瑞友宁对初级止血没有影响,VIII:C的半衰期非常短。如果需要迅速止血,且无法通过药物纠正缺陷时,我们建议使用含有因子VIII和完整vWF多聚体的浓缩物,但对于预防性治疗,可使用纯血管性血友病因子。

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