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获得性血管性血友病综合征中的胃肠道出血:替代治疗失败时大剂量免疫球蛋白的疗效

Gastro-intestinal bleeding in acquired von Willebrand's disease: efficacy of high-dose immuno-globulin where substitution treatments failed.

作者信息

White L A, Chisholm M

机构信息

Department of Haematology, Southampton General Hospital.

出版信息

Br J Haematol. 1993 Jun;84(2):332-4. doi: 10.1111/j.1365-2141.1993.tb03074.x.

Abstract

We report a case of acquired von Willebrand's disease with severe and persistent gastro-intestinal bleeding from multiple bleeding points. He received maximum substitution treatment including factor VIII concentrates, cryoprecipitate and platelets. He also received DDAVP. There was no change in the rate of blood loss. He was then treated with high-dose intravenous gamma-globulin and the blood loss stopped abruptly. He remained well for several months when bleeding recommenced and he again responded promptly to gamma-globulin. This is a useful maintenance treatment for patients with acquired von Willebrand's disease.

摘要

我们报告一例获得性血管性血友病患者,该患者出现多处出血点导致严重且持续性胃肠道出血。他接受了包括VIII因子浓缩物、冷沉淀和血小板在内的最大剂量替代治疗。他还接受了去氨加压素治疗。失血速率没有变化。随后他接受了大剂量静脉注射丙种球蛋白治疗,出血随即停止。在接下来的几个月里他情况良好,但后来再次出血,而他再次迅速对丙种球蛋白治疗产生反应。对于获得性血管性血友病患者,这是一种有效的维持治疗方法。

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