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病例报告:成功治疗一例具有不寻常特征的特发性获得性凝血因子 VIII 抑制剂。

Case report: successful treatment of an idiopathic acquired factor VIII inhibitor with unusual features.

作者信息

Makinen D, Mant M J

出版信息

Am J Med Sci. 1980 Nov-Dec;280(3):179-83. doi: 10.1097/00000441-198011000-00007.

Abstract

A patient with an idiopathic acquired factor VIII inhibitor is described. Unusual features of this patient's illness were measurable factor VIII (4 to greater than 20%) despite the presence of inhibitor, and continued spontaneous bleeding despite these factor VIII levels. Successful treatment on both a short-term and a long-term basis--with neutralizing doses of human factor VIII concentrate and with chlorambucil, respectively--is reported. It is concluded that in patients with this type of inhibitor factor VIII, assays may not provide a reliable prediction of the potential for bleeding and that chlorambucil therapy warrants further study.

摘要

本文描述了一名患有特发性获得性凝血因子VIII抑制物的患者。该患者病情的不寻常特征为:尽管存在抑制物,但仍可检测到凝血因子VIII(4%至大于20%),并且尽管有这些凝血因子VIII水平,仍持续出现自发性出血。分别报告了短期和长期成功治疗的情况,短期使用中和剂量的人凝血因子VIII浓缩物,长期使用苯丁酸氮芥。结论是,对于患有此类凝血因子VIII抑制物的患者,检测可能无法可靠预测出血可能性,苯丁酸氮芥治疗值得进一步研究。

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