Management of haemophilia B patients with inhibitors and anaphylaxis

被引：38

作者：

Warrier, I

机构：

[1] Wayne State Univ, Childrens Hosp Michigan, Div Hematol Oncol, Detroit, MI 48201 USA

[2] Wayne State Univ, Childrens Hosp Michigan, Hemophilia Treatment Ctr, Detroit, MI 48201 USA

来源：

HAEMOPHILIA | 1998年 / 4卷 / 04期

关键词：

hemophilia B; FIX inhibitor; anaphylaxis; rFVIIa;

D O I：

10.1046/j.1365-2516.1998.440574.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

The development of inhibitor antibodies is a serious complicaiton of haemophilia in young children. Occurrence of anaphylaxis at the time of inhibitor development. is a recently described complication unique to haemophilia B. Management of these inhibitor patients with allergy is complicated due to the absence of any readily available products for treatment of acute bleeding episodes. Clinical experience suggests that recombinant activated factor VII is the most appropriate and logical treatment for acute bleeding episodes in these patients. From the limited information available regarding immune tolerance induction (ITI) in these patients, it appears that ITI regimens have been only minimally successful and are associated with a high rate of complication (nephrotic syndrome).

引用

页码：574 / 576

页数：3

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