Successful treatment of congenital thrombotic thrombocytopenic purpura using the intermediate purity factor VIII concentrate BPL 8Y

被引：26

作者：

Lester, WA

Williams, MD

Allford, SL

Enayat, MS

Machin, SJ

机构：

[1] Birmingham Childrens Hosp, Dept Haematol, Birmingham, W Midlands, England

[2] UCL, Dept Haematol, London, England

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2002年 / 119卷 / 01期

关键词：

congenital thrombotic thrombocytopenic purpura; von Willebrand factor-cleaving protease; microangiopathy; intermediate purity factor VIII concentrate; fresh-frozen plasma;

D O I：

10.1046/j.1365-2141.2002.03809.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

There is increasing evidence that congenital thrombotic thrombocytopenic purpura (TTP) is caused by an absolute deficiency of von Willebrand factor-cleaving protease. The recent identification of this protease and the development of assays for its detection have enabled its quantification in a number of plasma products, including some commercial intermediate-purity plasma-derived factor VIII preparations. We report the successful, weekly prophylactic use of a commercial intermediate-purity plasma-derived factor VIII concentrate in the treatment of a 14-year-old girl with severe congenital TTP who had previously required transfusions of fresh-frozen plasma every 2 weeks from the age of 4 months.

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收藏

页码：176 / 179

页数：4

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