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甲型血友病的抑制剂研发:血管性血友病因子/凝血因子 VIII 浓缩物的作用

Inhibitor development in haemophilia A: the role of von Willebrand factor/factor VIII concentrates.

作者信息

Goudemand J

机构信息

Haematology Department, Lille University Hospital, Lille, France.

出版信息

Haemophilia. 2007 Dec;13 Suppl 5:47-51. doi: 10.1111/j.1365-2516.2007.01571.x.

Abstract

The presence of inhibitors that neutralize the function of factor VIII (FVIII) decreases the haemostatic efficacy of replacement clotting factor concentrate and increases morbidity among patients with haemophilia A. Certain genetic and environmental variables have been linked to a higher incidence of inhibitors. Conversely, the presence of von Willebrand factor (VWF) in some plasma-derived FVIII products may provide some measure of protection against inhibitor development, although the evidence is not conclusive. Clinical trials are needed to resolve this issue and determine the appropriate role of VWF-containing FVIII concentrates in the treatment of haemophilia A patients.

摘要

能够中和凝血因子VIII(FVIII)功能的抑制剂的存在会降低替代凝血因子浓缩物的止血效果,并增加甲型血友病患者的发病率。某些遗传和环境变量与抑制剂的较高发生率有关。相反,一些血浆源性FVIII产品中血管性血友病因子(VWF)的存在可能提供一定程度的保护,防止抑制剂的产生,尽管证据并不确凿。需要进行临床试验来解决这个问题,并确定含VWF的FVIII浓缩物在治疗甲型血友病患者中的适当作用。

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