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由于循环中的因子VIII抑制物导致的获得性因子VIII缺乏症。

Acquired factor VIIIC deficiency due to circulating factor VIIIC inhibitors.

作者信息

Kueh Y K, Koh D R, Chan T B, Han P

机构信息

Department of Medicine, National University Hospital, Singapore.

出版信息

Ann Acad Med Singap. 1988 Oct;17(4):589-94.

PMID:3146940
Abstract

The management of patients with Factor VIIIC inhibitor is frequently a therapeutic challenge. Treatment is often individualized. We describe 3 patients, nonhaemophilic adults, with bleeding diatheses caused by a circulating inhibitor to Factor VIIIC. One patient had long standing rheumatoid arthritis. The other two did not have any apparent underlying disease, although one of them had an antecedent antecedent phenylbutazone injection for arthralgias. Prednisolone was prescribed for two patients which resulted in rapid clinical improvement and the eventual normalization of Factor VIIIC activity. The role and rationale of immunosuppressive therapy in Factor VIIIC inhibitor is reviewed.

摘要

伴有Ⅷ因子抑制物的患者的管理常常是一项治疗挑战。治疗通常是个体化的。我们描述了3例非血友病成年患者,他们因循环中的Ⅷ因子抑制物而出现出血倾向。1例患者患有长期类风湿关节炎。另外2例没有任何明显的基础疾病,尽管其中1例曾因关节痛注射过保泰松。2例患者使用了泼尼松龙,临床症状迅速改善,Ⅷ因子活性最终恢复正常。本文综述了免疫抑制治疗在Ⅷ因子抑制物中的作用及理论依据。

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1
Acquired factor VIIIC deficiency due to circulating factor VIIIC inhibitors.由于循环中的因子VIII抑制物导致的获得性因子VIII缺乏症。
Ann Acad Med Singap. 1988 Oct;17(4):589-94.
2
[Acquired hemophilia A due to factor VIIIC inhibitor: detection of circulating immune complexes containing factor VIIIC but not factor VIIIR].
Schweiz Med Wochenschr. 1983 Oct 8;113(40):1456-9.
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Factor VIIIc inhibitors: an automated assay.凝血因子VIIIc抑制剂:一种自动化检测方法。
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[Acquired factor VIIIc inhibitors. Two cases and a review of the literature].[获得性凝血因子VIIIc抑制剂。两例病例及文献综述]
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Acquired hemophilia in the patient suffering from rheumatoid arthritis: case report.类风湿关节炎患者获得性血友病:病例报告
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Identification of six functional clotting factor VIII:C epitopes by analysis of cross-reactive public idiotypes in murine monoclonal VIII:C inhibitors.通过分析鼠单克隆VIII:C抑制剂中的交叉反应性公共独特型鉴定六种功能性凝血因子VIII:C表位
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