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获得性血管性血友病。关于定量和定性因子VIII紊乱的证据。

Acquired von Willebrand's disease. Evidence for a quantitative and qualitative factor VIII disorder.

作者信息

Joist J H, Cowan J F, Zimmerman T S

出版信息

N Engl J Med. 1978 May 4;298(18):988-91. doi: 10.1056/NEJM197805042981802.

Abstract

To define further the factor VIII abnormality in acquired von Willebrand's disease, we performed immunoelectrophoresis of factor VIII antigen, as well as quantitative measurements of the antigen, factor VIII procoagulant activity and von Willebrand factor activity on plasma from an affected 57-year-old man who also had a poorly differentiated lymphocytic lymphoma. No evidence for an inhibitor against factor VIII procoagulant activity or von Willebrand factor activity was detected, but immunoelectrophoresis showed none of the less anodic forms of factor VIII antigen. There were concomitant decreases in total antigen (0.19 U per milliliter) and von Willebrand factor levels (0.12 U per milliliter). Factor VIII-procoagulant activity was borderline low (0.45 U per milliliter). Correction of both the abnormal immunoelectrophoresis pattern and the quantitative abnormalities followed radiotherapy of the lymphoma. The factor VIII abnormalities might have resulted from binding or destruction of theless anodic forms of factor VIII antigen by the malignant lymphocytes.

摘要

为进一步明确获得性血管性血友病中凝血因子VIII异常的情况,我们对一名57岁患病男性的血浆进行了凝血因子VIII抗原免疫电泳,以及该抗原、凝血因子VIII促凝活性和血管性血友病因子活性的定量检测。该男性还患有低分化淋巴细胞淋巴瘤。未检测到针对凝血因子VIII促凝活性或血管性血友病因子活性的抑制剂,但免疫电泳显示不存在凝血因子VIII抗原的阳极较弱形式。总抗原(每毫升0.19 U)和血管性血友病因子水平(每毫升0.12 U)同时降低。凝血因子VIII促凝活性临界偏低(每毫升0.45 U)。淋巴瘤放疗后,异常免疫电泳图谱和定量异常均得到纠正。凝血因子VIII异常可能是由于恶性淋巴细胞结合或破坏了凝血因子VIII抗原的阳极较弱形式所致。

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