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Coagulation Disorders Haemophilia
been switched to different products during treatment. This result Numerous patient-related risk factors are thought to be associated with
confirms previous findings in 73 haemophilia patients where the ratio for inhibitor development. For example, a family history of inhibitor
switching to another product or remaining on the same product was development and the presence of specific gene mutations have been
0.4.
42
Similarly, no increased risk of inhibitor development has been linked to a higher risk of inhibitors. Also, the presence of inhibitors in
reported after switching FVIII products in previously treated patients.
43,44
African-Americans has been reported to be twice that in Caucasians.
There are also treatment-related risk factors: in several studies a high-
intensity treatment regimen was reported to increase the risk of inhibitor
development. Prophylaxis has been found to reduce the development of
Prophylaxis has been found to
inhibitors and is recommended for children with severe haemophilia A.
The effect of the individual factor used on inhibitor incidence needs
reduce the development of inhibitors
further study. Some studies report that rFVIII has a significantly higher risk
and is recommended for children
of inhibitor development compared with pdFVIII, while others report no
significant risk. Moreover, individual FVIII concentrates have been
with severe haemophilia A.
associated with a wide range of inhibitor development rates. It is yet to
be confirmed whether FVIII product choice in general has a significant
effect on the development of inhibitors in previously untreated patients.
Current evidence points to the development of inhibitors as a
Summary multifactorial process that both is polygenic and involves several
The development of inhibitors in children with haemophilia A presents a environmental factors. Further investigation will help to better define the
major problem in terms of both the cost and the success of treatment. interactions between these risk factors and immune responses. ■
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10 EUROPEAN HAEMATOLOGY 2007
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