Increased inhibitor incidence in severe haemophilia A since 1990 attributable to more low titre inhibitors
- van den Berg, H. Marijke
- Hashemi, S. Mojtaba
- Fischer, Kathelijn
- Petrini, Pia
- Ljung, Rolf
- Rafowicz, Anne
- Carcao, Manuel
- Auerswald, Günter
- Kurnik, Karin
- Kenet, Gili
- Santagostino, Elena
DOIAbstract
Many studies have reported an increased incidence of inhibitors in previously untreated patients (PUPs) with severe haemophilia A after the introduction of recombinant products. It was the objective of this study to investigate whether the inhibitor incidence has increased between 1990 and 2009 in an unselected cohort of PUPs with severe haemophilia A (FVIII<1 %). Patients were consecutively recruited from 31 haemophilia treatment centres in 16 countries and followed until 50 exposure days or until inhibitor development. Inhibitor development was studied in five-year birth cohorts comparing cumulative incidences. Furthermore the risk for inhibitor development per fiveyear birth cohort was studied using multivariable Cox regression, adjustin...
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