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获得性血友病合并Ⅲ型血管性血友病:高纯度血管性血友病因子和重组凝血因子VIIa治疗成功

Acquired haemophilia in association with type III von Willebrand's disease: successful treatment with high purity von Willebrand's factor and recombinant factor VIIa.

作者信息

Majumdar G, Phillips J K, Lavallee H, Savidge G F

机构信息

Haemophilia Reference Centre, St Thomas' Hospital, London, UK.

出版信息

Blood Coagul Fibrinolysis. 1993 Dec;4(6):1035-7.

PMID:8148477
Abstract

A patient with autosomal dominant (Type III) von Willebrand's disease (vWd) developed acquired haemophilia post-operatively, possibly due to exposure to amoxycillin. She refused porcine factor VIII (pFVIII) on religious grounds and was managed successfully with recombinant activated factor VII (rFVIIa) together with highly purified von Willebrand factor concentrate (vWf-VHP). In patients with acquired haemophilia rFVIIa appears to be a suitable agent for symptomatic management.

摘要

一名患有常染色体显性(III型)血管性血友病(vWd)的患者术后发生了获得性血友病,可能是由于接触阿莫西林所致。她出于宗教原因拒绝使用猪源因子VIII(pFVIII),通过重组活化因子VII(rFVIIa)联合高纯度血管性血友病因子浓缩物(vWf-VHP)成功得到治疗。对于获得性血友病患者,rFVIIa似乎是一种合适的对症治疗药物。

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