Factor VIII inhibitors in haemophiliacs: a single-centre experience over 34 years, 1964-97.
A retrospective study of the natural history of factor VIII inhibitors in haemophilia A patients experienced in a single comprehensive haemophilia centre over three decades is reported. 431 haemophilia A patients of all severities have been followed-up for a total of 5626 patient-years. The frequency of inhibitors was 10% in the severe haemophilia A patients and 37% occurred in children <10 years. The majority of the patients received several products before developing the inhibitors. 59% of patients had <50 exposure days and 48% were high responders (>5 BU). An 8-year (1987-95) inhibitor-free period was seen during which all previously untreated patients were treated with an intermediate-purity factor VIII concentrate. A moderate haemophiliac with a missense mutation that has not been described in association with inhibitor is reported. Six HIV-positive patients preserved their antibody response to factor VIII even at the advanced stage of their disease.
Yee
T T
TT
Katharine Dormandy Haemophilia Centre, Department of Haematology, Royal Free Hospital, London.
Pasi
K J
KJ
Lilley
P A
PA
Lee
C A
CA
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016913:Blood Component Transfusion / Q000379:methods / Q000706:statistics & numerical data; D002648:Child; D002675:Child, Preschool; D005006:Ethnicity; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000235:genetics / Q000627:therapeutic use; D015658:HIV Infections / Q000209:etiology; D006467:Hemophilia A / Q000188:drug therapy / Q000453:epidemiology* / Q000235:genetics; D006801:Humans; D007223:Infant; D008131:London / Q000453:epidemiology; D012189:Retrospective Studies
D005169:Factor VIII