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获得性血管性血友病综合征和埃勒斯-当洛综合征伴发胃肠道出血。

Acquired von Willebrand's syndrome and Ehlers-Danlos syndrome presenting with gastro-intestinal bleeding.

作者信息

Clough V, MacFarlane I A, O'Connor J, Wood J K

出版信息

Scand J Haematol. 1979 Apr 4;22(4):305-10. doi: 10.1111/j.1600-0609.1979.tb00424.x.

Abstract

The clinical and laboratory findings in a patient with severe gastro-intestinal bleeding and the Ehlers-Danlos syndrome are described. Coagulation studies and a lack of history of previous haemorrhage were consistent with a diagnosis of acquired von Willebrand's syndrome. His response to treatment with blood transfusion, Factor VIII, cimetidine, tranexamic acid and cyclophosphamide is described. Family studies revealed other members with Ehlers-Danlos syndrome but normal coagulation.

摘要

本文描述了一名患有严重胃肠道出血的埃勒斯-当洛综合征患者的临床和实验室检查结果。凝血研究以及既往无出血史与获得性血管性血友病综合征的诊断相符。文中还描述了他接受输血、凝血因子 VIII、西咪替丁、氨甲环酸和环磷酰胺治疗后的反应。家族研究显示,其他患有埃勒斯-当洛综合征的家庭成员凝血功能正常。

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