title: Epidemiology of inhibitors in haemophilia A.
abstract: One of the most serious complications of the treatment of haemophilia A is the development of inhibitors. Former studies mostly considered the prevalence of inhibitor development, thus underestimating its true risk. Prevalences ranged widely (7-18%) probably due to the populations studied and the study design. Recent prospective previously untreated patients (PUP) studies were more comparable because of similar study designs. Eight PUP studies regarding the incidence of factor VIII inhibitors were analyzed: The inhibitor incidences (Independent of severity of haemophilia) ranged from 18.4 to 28%. Evaluating only severe haemophiliacs (factor VIII < 2%) significantly higher incidences were found. After 9-36 exposure days (as medians inhibitor development occurred at 0.8-3.3 years of age (as medians).
authors.affiliation: Department of Haematology and Oncology, J.W. Goethe University Hospital, Frankfurt, Germany.
authors.forename: W
authors.initials: W
authors.lastname: Kreuz
authors.forename: C
authors.initials: C
authors.lastname: Escuriola-Ettingshausen
authors.forename: I
authors.initials: I
authors.lastname: Martinez-Saguer
authors.forename: T
authors.initials: T
authors.lastname: Güngör
authors.forename: B
authors.initials: B
authors.lastname: Kornhuber
mesh_terms: D005169:Factor VIII / Q000009:adverse effects* / Q000276:immunology; D006467:Hemophilia A / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology; D006801:Humans; D007518:Isoantibodies / Q000276:immunology*; D015995:Prevalence
chemical_list: D007518:Isoantibodies; D005169:Factor VIII