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一名可能同时患有甲型血友病和血管性血友病患者体内的凝血因子VIII:C抑制剂。

An inhibitor to factor VIII:C in a patient with possible combined haemophilia A and von Willebrand's disease.

作者信息

Taylor L D, Dean F L, Tiedemann K, Ekert H

出版信息

Thromb Haemost. 1986 Apr 30;55(2):158-61.

PMID:3087000
Abstract

A factor VIII inhibitor has been found in a patient with an unusual combination of factor VIII-related properties. The inhibitor is directed specifically against the clotting activity (VIII:C) of the factor VIII complex. It behaves in a similar fashion to high responding inhibitors of factor VIII seen in haemophilia A patients and it was characterised as an immunoglobulin of the IgG class. Laboratory results from the patient and his family show considerable variation of factor VIII-related properties between various individuals. Overall, the data suggests the co-existence of haemophilia A and von Willebrand's disease in the family and the presence of both diseases in the patient.

摘要

在一名具有不寻常的VIII因子相关特性组合的患者中发现了一种VIII因子抑制剂。该抑制剂特异性针对VIII因子复合物的凝血活性(VIII:C)。其行为方式与A型血友病患者中所见的高反应性VIII因子抑制剂相似,并且被鉴定为IgG类免疫球蛋白。该患者及其家族的实验室结果显示,不同个体之间VIII因子相关特性存在相当大的差异。总体而言,数据表明该家族中同时存在A型血友病和血管性血友病,且该患者同时患有这两种疾病。

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