FRENCH PREVIOUSLY UNTREATED PATIENTS WITH SEVERE HEMOPHILIA-A AFTER EXPOSURE TO RECOMBINANT FACTOR-VIII - INCIDENCE OF INHIBITOR AND EVALUATION OF IMMUNE TOLERANCE

Citation
C. Rothschild et al., FRENCH PREVIOUSLY UNTREATED PATIENTS WITH SEVERE HEMOPHILIA-A AFTER EXPOSURE TO RECOMBINANT FACTOR-VIII - INCIDENCE OF INHIBITOR AND EVALUATION OF IMMUNE TOLERANCE, Thrombosis and haemostasis, 80(5), 1998, pp. 779-783
Citations number
32
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Hematology,"Peripheal Vascular Diseas
Journal title
ISSN journal
0340-6245
Volume
80
Issue
5
Year of publication
1998
Pages
779 - 783
Database
ISI
SICI code
0340-6245(1998)80:5<779:FPUPWS>2.0.ZU;2-L
Abstract
Fifty French previously untreated patients with severe hemophilia A (f actor Vm < 1%), treated with only one brand of recombinant factor vm ( rFVIII), were evaluated for inhibitor development, assessment of risk factors and outcome of immune tolerance regimen. The median period on study was 32 months (range 9-74) since the first injection of rFVIII. Fourteen patients (28%) developed an inhibitor, four of whom (8%) with a high titer (greater than or equal to 10 BU). All inhibitor patients but one continued to receive rFVIII: either for on-demand treatment o r for immune tolerance regimen (ITR). Among these patients, inhibitor was transient in 2 (4%), became undetectable in 6 and was still presen t in 6. The prevalence of inhibitor was 12%. Presence of intron 22 inv ersion was found to be a risk factor for inhibitor development Immune tolerance was difficult to achieve in our series despite a follow-up p eriod of 16 to 30 months: immune tolerance was complete in only one ou t of the 3 patients undergoing low dose ITR and in one out of the 5 pa tients with high dose ITR.