[中心静脉穿刺置管术成功治疗难治性获得性血友病A]

[Refractory acquired hemophilia A successfully treated with CVP].

作者信息

Kaneko Hitomi, Okada Noriko, Matsui Yusuke, Nishizawa Masatoshi, Watanabe Mitsumasa, Miura Yasuo, Tsudo Mitsuru

机构信息

Department of Hematology, Osaka Red Cross Hospital, Japan.

出版信息

Rinsho Ketsueki. 2009 Feb;50(2):110-2.

PMID:19265305

Abstract

A 44-year-old woman was referred to our hospital for massive subcutaneous and intramuscular hemorrhage. Prolonged APTT, low factor VIII activity and factor VIII inhibitor with high titer (30 BU/ml) were observed, confirming the diagnosis of acquired factor VIII inhibitor. Although treated with methylprednisolone, she relapsed after a month. Subsequently, she was treated with three courses of CVP (cyclophosphamide, vincristine, prednisolone) therapy, combined with recombinant activated factor VII. The activity of factor VIII was normalized one week after starting CVP, and the inhibitor disappeared 13 months later. She has maintained complete remission for 26 months without recurrence to date. CVP therapy is very effective against refractory acquired factor VIII inhibitor.

摘要

一名44岁女性因大量皮下及肌肉内出血被转诊至我院。观察到活化部分凝血活酶时间（APTT）延长、凝血因子VIII活性降低以及高滴度（30 BU/ml）的凝血因子VIII抑制剂，确诊为获得性凝血因子VIII抑制剂。尽管接受了甲泼尼龙治疗，但1个月后病情复发。随后，她接受了三个疗程的CVP（环磷酰胺、长春新碱、泼尼松龙）治疗，并联合重组活化凝血因子VII。开始CVP治疗1周后，凝血因子VIII活性恢复正常，13个月后抑制剂消失。迄今为止，她已维持完全缓解26个月，未复发。CVP疗法对难治性获得性凝血因子VIII抑制剂非常有效。