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一名轻度血友病患者体内的凝血因子VIII抗体

Factor VIII antibody in a patient with mild haemophilia.

作者信息

Bovill E G, Burns S L, Golden E A

出版信息

Br J Haematol. 1985 Oct;61(2):323-8. doi: 10.1111/j.1365-2141.1985.tb02832.x.

Abstract

We present the rare occurrence of an inhibitor of factor VIII procoagulant arising in a patient with mild haemophilia A and rheumatoid arthritis. The inhibitor was transient and behaved like a low titre, type II factor VIII procoagulant inhibitor similar to previously reported cases (Biggs et al, 1972b). In vitro studies confirmed the type II-like interaction of this inhibitor with the factor VIII procoagulant molecule. Factor VIII procoagulant antigen level was equal to the factor VIII procoagulant activity, which excluded dysproteinaemia as the cause. This patient's HLA type has no known association with abnormal immune responsiveness or autoimmune disease, and his clinical course as well as in vitro studies were similar to the eight previously reported cases of factor VIII procoagulant inhibitors arising in mild haemophilia A.

摘要

我们报告了1例患轻度甲型血友病和类风湿性关节炎的患者出现凝血因子Ⅷ促凝剂抑制物的罕见病例。该抑制物为短暂性,表现为低滴度Ⅱ型凝血因子Ⅷ促凝剂抑制物,与先前报道的病例相似(比格斯等人,1972年b)。体外研究证实了该抑制物与凝血因子Ⅷ促凝剂分子的Ⅱ型样相互作用。凝血因子Ⅷ促凝剂抗原水平与凝血因子Ⅷ促凝剂活性相等,排除了异常蛋白血症作为病因。该患者的人类白细胞抗原(HLA)类型与已知的异常免疫反应性或自身免疫性疾病无关,其临床病程以及体外研究与先前报道的8例轻度甲型血友病患者出现凝血因子Ⅷ促凝剂抑制物的病例相似。

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