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产后VIII因子抑制剂

Factor VIII inhibitor postpartum.

作者信息

Michiels J J, Bosch L J, van der Plas P M, Abels J

出版信息

Scand J Haematol. 1978 Feb;20(2):97-107. doi: 10.1111/j.1600-0609.1978.tb02434.x.

Abstract

Acquired factor VIII deficiency in women postpartum due to a factor VIII inhibitor is rare and the etiology is unknown. In this study a case report and a review of the literature are given. The haemorrhagic diathesis resembles classic haemophilia, with the exception that ecchymoses and tissue bleeding occur more frequently. The potency of the inhibitor may vary from weak to strong and the inactivation of factor VIII coagulant activity (factor VIII-C) by the inhibitor is of a non-linear type. Severe bleeding has been fatal in a few cases, but factor VIII concentrate substitution has usually been successful without anamnestic response of inhibitor activity. There is no convincing evidence that immunosuppression is effective, also because the natural history of the disease is characterised by a spontaneous disappearance of the factor VIII-C inhibitor. Treatment of bleeding symptoms with factor VIII concentrate should therefore not be reserved for life threatening haemorrhages only.

摘要

产后女性因 VIII 因子抑制物导致获得性 VIII 因子缺乏症较为罕见,病因不明。本研究给出了一例病例报告并对文献进行了综述。出血素质类似于典型血友病,不同之处在于瘀斑和组织出血更为常见。抑制物的效力可能从弱到强不等,且抑制物对 VIII 因子凝血活性(VIII 因子 -C)的灭活呈非线性。少数情况下严重出血是致命的,但 VIII 因子浓缩物替代治疗通常成功,且抑制物活性无记忆反应。没有令人信服的证据表明免疫抑制有效,这也是因为该疾病的自然病程特征为 VIII 因子 -C 抑制物自发消失。因此,不应仅在危及生命的出血时才使用 VIII 因子浓缩物治疗出血症状。

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