Epidemiological aspects of inhibitor development redefine the clinical importance of inhibitors.
Inhibitor development is a serious complication of treatment with coagulation products. Presently, 25-30% of all newly diagnosed patients with severe haemophilia A are diagnosed with inhibitors. An increasing number of genetic and non-genetic risk factors have been reported to be involved, although the impact of them in understanding the aetiology is still limited. Much attention has been focused on factor VIII products, but more recent studies show very little, if any, difference between class plasma and recombinant factor VIII products. More intensive treatment and higher dosing are probably more important factors. More than 10% of the inhibitors diagnosed in the last decade are of low titre. A first goal should be to understand their importance. It is argued that the impact of different risk factors should be studied in high-titre inhibitors to prevent dilution by non-significant low-titre inhibitors.
Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, the Netherlands.
H M
HM
van den Berg
D005169:Factor VIII / Q000276:immunology* / Q000627:therapeutic use*; D006467:Hemophilia A / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology*; D006801:Humans; D015994:Incidence; D007518:Isoantibodies / Q000097:blood / Q000276:immunology*; D011994:Recombinant Proteins / Q000276:immunology / Q000627:therapeutic use
D007518:Isoantibodies; D011994:Recombinant Proteins; D005169:Factor VIII
PUPs; children; inhibitors; low-titre inhibitors; severe haemophilia