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去氨加压素治疗获得性血友病A:病例报告及文献复习

DDAVP in acquired hemophilia A: case report and review of the literature.

作者信息

Mudad R, Kane W H

机构信息

Department of Medicine, Duke University Medical Center, Durham, North Carolina 27710.

出版信息

Am J Hematol. 1993 Aug;43(4):295-9. doi: 10.1002/ajh.2830430413.

Abstract

One patient with an acquired factor VIII inhibitor is reported in which an acute lower intestinal hemorrhage was successfully managed using Desmopressin (DDAVP). The patient initially had a factor VIII level of 10% with a inhibitor titer of 1.9 Bethesda units. Following administration of DDAVP the factor VIII level rose to 86% and there was a decrease in the number and volume of bloody stools. The inhibitor disappeared following treatment with corticosteroids, however the patient ultimately expired due to complications of ischemic colitis. This case and 21 previously reported cases of acquired hemophilia treated with DDAVP are reviewed. The data support a role for DDAVP in the treatment of non life threatening hemorrhage in patients with acquired hemophilia and low titer factor VIII inhibitors (< 5 Bethesda units or factor VIII > or = 5%).

摘要

本文报告了1例获得性Ⅷ因子抑制物患者,该患者因急性下消化道出血,使用去氨加压素(DDAVP)成功治愈。患者最初Ⅷ因子水平为10%,抑制物滴度为1.9贝塞斯达单位。给予DDAVP后,Ⅷ因子水平升至86%,便血的数量和量均减少。使用皮质类固醇治疗后抑制物消失,但患者最终因缺血性结肠炎并发症死亡。本文回顾了该病例以及之前报道的21例使用DDAVP治疗获得性血友病的病例。数据支持DDAVP在治疗获得性血友病且Ⅷ因子抑制物滴度较低(<5贝塞斯达单位或Ⅷ因子>或=5%)的非危及生命的出血中发挥作用。

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