Table. Inhibition of fVIII activity in mild hemophilia a patients' plasma.
fVIII Mutation* . | fVIII Activity (IU/ml)† . | LE2E9 Inhibition (%)‡ . |
---|---|---|
Arg1689Leu | 0.04 | >70 |
Arg1749His | 0.39 | >70 |
Gly1750Arg | 0.38 | >70 |
Ala1824Val | 0.16 | >70 |
Asp1825Gly | 0.17 | >70 |
His1961Tyr | 0.24 | >70 |
Arg1966Gln | 0.08 | >70 |
Met2010Ile | 0.04 | >70 |
Ser2011Asn | 0.23 | >70 |
Val2016Ala | 0.05 | >70 |
Asn2019ser | 0.13 | >70 |
Leu2052Phe | 0.23 | >70 |
Asp2074Gly | 0.13 | >70 |
Thr2086Ile | 0.08 | >70 |
Ile2098Ser | 0.20 | >70 |
Phe2101Leu | 0.06 | >70 |
Asn2129Ser | 0.20 | >70 |
Arg2150His | 0.03 | 0 |
Pro2153Gln | 0.02 | 65 |
Arg2159Leu | 0.16 | >70 |
Trp2229Cys | 0.02 | >70 |
Gln2246Arg | 0.08 | >70 |
fVIII Mutation* . | fVIII Activity (IU/ml)† . | LE2E9 Inhibition (%)‡ . |
---|---|---|
Arg1689Leu | 0.04 | >70 |
Arg1749His | 0.39 | >70 |
Gly1750Arg | 0.38 | >70 |
Ala1824Val | 0.16 | >70 |
Asp1825Gly | 0.17 | >70 |
His1961Tyr | 0.24 | >70 |
Arg1966Gln | 0.08 | >70 |
Met2010Ile | 0.04 | >70 |
Ser2011Asn | 0.23 | >70 |
Val2016Ala | 0.05 | >70 |
Asn2019ser | 0.13 | >70 |
Leu2052Phe | 0.23 | >70 |
Asp2074Gly | 0.13 | >70 |
Thr2086Ile | 0.08 | >70 |
Ile2098Ser | 0.20 | >70 |
Phe2101Leu | 0.06 | >70 |
Asn2129Ser | 0.20 | >70 |
Arg2150His | 0.03 | 0 |
Pro2153Gln | 0.02 | 65 |
Arg2159Leu | 0.16 | >70 |
Trp2229Cys | 0.02 | >70 |
Gln2246Arg | 0.08 | >70 |
Mutations in the fVIII gene were detected according to reference 9.
fVIII levels in the patient's plasma were measured in a fVIII chromogenic assay.
Equal volumes of LE2E9 (20 μg/ml) and of plasma were incubated for 1 hour at 37°C. The residual fVIII activity was measured in a fVIII chromogenic assay and expressed in percentage of the activity obtained in the absence of antibody. Experiments were repeated at least twice with similar results.