Recommendations for the treatment of factor VIII inhibitors: From the UK Haemophilia Centre directors' organisation inhibitor working party

被引:43
作者
Hay, CRM
Colvin, BT
Ludlam, CA
Hill, FGH
Preston, FE
机构
[1] UNIV LONDON,ROYAL LONDON HOSP,DEPT HAEMATOL,LONDON E1 1BB,ENGLAND
[2] ROYAL EDINBURGH & ASSOCIATED HOSP,DEPT HAEMATOL,EDINBURGH EH8 9AG,MIDLOTHIAN,SCOTLAND
[3] CHILDRENS HOSP,DEPT HAEMATOL,BIRMINGHAM B16 8ET,W MIDLANDS,ENGLAND
[4] UNIV SHEFFIELD,ROYAL HALLAMSHIRE HOSP,DEPT HAEMATOL,SHEFFIELD S10 2JF,S YORKSHIRE,ENGLAND
关键词
factor VIII inhibitors; haemophilia; therapeutic recommendations;
D O I
10.1097/00001721-199603000-00005
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
A strategy is described for the initial detection, management and elimination of factor WI inhibitors arising in patients with congenital and acquired haemophilia A. It is suggested that children with severe haemophilia A should be screened every 3 months up to the age of 10 years for inhibitors using the Bethesda method Factor Vm inhibitors arising in these patients should be abolished using immune-tolerance induction wherever possible. Such regimes should be started as early as possible, preferably when the inhibitor titre is < 10 Bethesda Units (BU)/ml, and should not be interrupted High-intensity regimes are recommended for patients whose inhibitors exceed 10 BU. Autoantibodies to factor Vm giving rise to acquired haemophilia should be abolished using high-dose immunoglobulin or conventional immunosuppression. The choice of haemostatic agent for the treatment of severe bleeding should be based upon the clinical circumstances and the current inhibitor value, measured using both human and porcine factor VIII in the Bethesda assay. The past anamnestic response should also be considered when choosing treatment for minor bleeding episodes.
引用
收藏
页码:134 / 138
页数:5
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