TY - JOUR
T1 - Analysis of factor VIII inhibitors in a haemophilia A patient with an Arg(593)-> Cys mutation using phage display
AU - Bril, Wendy S.
AU - Turenhout, Ellen A. M.
AU - Kaijen, Paul H. P.
AU - van den Brink, Edward N.
AU - Koopman, Maria M. W.
AU - Peters, Marjolein
AU - Voorberg, Jan
PY - 2002
Y1 - 2002
N2 - 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
AB - 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
U2 - https://doi.org/10.1046/j.1365-2141.2002.03856.x
DO - https://doi.org/10.1046/j.1365-2141.2002.03856.x
M3 - Article
C2 - 12406075
SN - 0007-1048
VL - 119
SP - 393
EP - 396
JO - British journal of haematology
JF - British journal of haematology
IS - 2
ER -