Analysis of factor VIII inhibitors in a haemophilia A patient with an Arg(593)-> Cys mutation using phage display

Wendy S. Bril, Ellen A. M. Turenhout, Paul H. P. Kaijen, Edward N. van den Brink, Maria M. W. Koopman, Marjolein Peters, Jan Voorberg

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Abstract

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
Original languageEnglish
Pages (from-to)393-396
JournalBritish Journal of Haematology
Volume119
Issue number2
DOIs
Publication statusPublished - 2002

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