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Analysis of factor VIII inhibitors in a haemophilia A patient with an Arg593→Cys mutation using phage display

被引：8
作者：
Bril, WS
Turenhout, EAM
Kaijen, PHP
van den Brink, EN
Koopman, MMW
Peters, M
Voorberg, J
机构：
[1] CLB, Sanquin Res, Dept Plasma Prot, NL-1066 CX Amsterdam, Netherlands
[2] Univ Amsterdam, Acad Med Ctr, Dept Vasc Med, NL-1105 AZ Amsterdam, Netherlands
[3] Univ Amsterdam, Acad Med Ctr, Emma Childrens Hosp, Dept Paediat, NL-1105 AZ Amsterdam, Netherlands
来源：
BRITISH JOURNAL OF HAEMATOLOGY | 2002年 / 119卷 / 02期
关键词：
factor VIII inhibitors; haemophilia A; phage display; B lymphocyte; immunoglobulin repertoire;
D O I：
10.1046/j.1365-2141.2002.03856.x
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
We characterized anti-factor VIII antibodies in a mild haemophilia A patient with an Arg(593)-->Cys mutation in the A2 domain, using V gene phage-display technology. All isolated single-chain variable-domain antibody fragments were directed against residues Arg(484)-Ile(508), a binding site for factor VIII inhibitors in the A2 domain. After a further period of replacement therapy, a transient rise in inhibitor titre was observed. These antibodies were directed against the A2 domain. Activation of a pre-existing pool of B cells, which express antibodies against residues Arg(484)-Ile(508), could explain the rapid anamnestic response.
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收藏
页码：393 / 396
页数：4
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