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获得性血管性血友病综合征合并胃肠道血管发育异常:一例报告

Acquired von Willebrand's disease associated with gastrointestinal angiodysplasia: a case report.

作者信息

Gupta P K, Kannan M, Chatterjee T, Dixit A, Mahapatra M, Choudhry V P, Saxena R

机构信息

Department of Haematoloy, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Haemophilia. 2006 Jul;12(4):452-5. doi: 10.1111/j.1365-2516.2006.01301.x.

Abstract

Acquired von Willebrand syndrome (AVWS) is a rare and probably underestimated bleeding disorder which mimics most of the clinical symptoms and laboratory features of hereditary von Willebrand disease (VWD) in patients devoid of both personal and family history of bleeding diathesis. In this study, we present a case of 55 yrs male patient, presented with gastrointestinal bleeds since three years, diagnosed to have AVWS with inhibitors. From this study it is concluded that AVWS is rare and it is important to diagnose this bleeding disorder so that appropriate treatment with plasmapheresis and IV:Ig can effectively correct the haemostatic defect and manage severe bleeding in these patients.

摘要

获得性血管性血友病综合征(AVWS)是一种罕见且可能被低估的出血性疾病,在没有个人及家族出血素质病史的患者中,它模拟了遗传性血管性血友病(VWD)的大多数临床症状和实验室特征。在本研究中,我们报告了一例55岁男性患者,该患者3年来一直有胃肠道出血,被诊断为患有伴有抑制剂的AVWS。从本研究可以得出结论,AVWS很罕见,诊断这种出血性疾病很重要,以便通过血浆置换和静脉注射免疫球蛋白进行适当治疗,能够有效纠正止血缺陷并处理这些患者的严重出血情况。

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