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[获得性血友病。关于一例致死病程的病例报告]

[Acquired hemophilia. Apropos of a case report with a fatal course].

作者信息

de Moerloose P, Tissot J D, Reber G, Schifferli J, Vogel J, Bouvier C A

机构信息

Département de médecine, Hôpital cantonal universitaire, Genève.

出版信息

Schweiz Med Wochenschr. 1988 Oct 22;118(42):1542-5.

PMID:3144037
Abstract

We report the case of an 86-year-old female with an unremarkable past history who suddenly developed severe and generalized bleeding. Laboratory work-up revealed prolonged aPTT, a factor VIII level of 7% and the presence of a factor VIII inhibitor. In spite of whole blood, factor VIII concentrates and immunoglobulin administration, the patient died of multiple hemorrhages on the third day after admission. At autopsy no causal or associated pathology could be found. Chromatographic separation of plasma immunoglobulins showed that the anti-factor VIII activity was associated with the patient's IgG. This inhibition was partially corrected in vitro by injectable immunoglobulin preparation. The case report stresses the discrepancy between the measured levels of inhibitor and the severity of the bleeding; our in vitro results may shed light on the mode of action of intravenous immunoglobulins.

摘要

我们报告了一例86岁女性病例,该患者既往史无特殊,却突然出现严重的全身性出血。实验室检查显示活化部分凝血活酶时间(aPTT)延长,凝血因子VIII水平为7%,且存在凝血因子VIII抑制剂。尽管给予了全血、凝血因子VIII浓缩物和免疫球蛋白治疗,但患者在入院后第三天死于多处出血。尸检未发现病因或相关病理改变。血浆免疫球蛋白的色谱分离显示,抗凝血因子VIII活性与患者的IgG相关。这种抑制作用在体外可被注射用免疫球蛋白制剂部分纠正。该病例报告强调了抑制剂检测水平与出血严重程度之间的差异;我们的体外实验结果可能有助于阐明静脉注射免疫球蛋白的作用方式。

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