Research : HLA and Haemophilia A inhibitors
Immunogenetic background of inhibitor formation in Haemophilia A
Anni Pavlova (Frankfurt)Jörg Schröder (Würzburg)
Inhibitor formation affects 20-30% of patients with severe haemophilia A and represents the major complication of substitution therapy with factor VIII concentrates. Since prophylactic treatment is not possible in those patients they are at risk for progressive joint damage. The pathomechanism of inhibitor formation is only partly understood. We have shown, that beside the FVIII mutation genetic determinants of the immune response genes e. g. HLA, play an important role for inhibitor formation (Oldenburg et al. 1997, Oldenburg et al. 2004). In the present study a cohort of 300 genetically characterized patients with severe haemophilia A, half with and half without inhibitors have been gathered to further study the influence of the HLA system on the risk of inhibitor formation.