Some recent developments regarding arthropathy and inhibitors in haemophilia.
In this paper, the most recent developments on arthropathy and inhibitors in haemophilia are reviewed. Firstly, early long-term prophylaxis is strongly recommended in children with severe haemophilia. Secondly, in joint bleeds not responding to substitution of factor VIII to normal factor VIII levels angiographic embolization might be considered as a promising therapeutic option. Thirdly, recent data indicate disruption of normal development for adolescents with inhibitors. Fourthly, some case reports indicate the benefit of secondary prophylaxis with recombinant factor VIIa (rFVIIa) or factor eight inhibitor bypassing activity (FEIBA) in patients with severe haemophilia A and inhibitors. Finally, surgical procedures in haemophilia patients with factor inhibitors can be accomplished safely and effectively with FEIBA and/or rFVIIa, although the risk of complications is higher than in haemophilia patients without inhibitors.
Rodriguez-Merchan
E C
EC
Department of Orthopaedics and Haemophilia Unit, La Paz University Hospital, Madrid, Spain. rmerchan@arrakis.es
D000293:Adolescent; D001323:Autoantibodies / Q000097:blood; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D002648:Child; D005169:Factor VIII / Q000276:immunology; D015942:Factor VIIa / Q000627:therapeutic use*; D006395:Hemarthrosis / Q000097:blood / Q000517:prevention & control* / Q000523:psychology; D006467:Hemophilia A / Q000188:drug therapy* / Q000276:immunology / Q000523:psychology; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D011788:Quality of Life; D011994:Recombinant Proteins / Q000627:therapeutic use
D001323:Autoantibodies; D001779:Blood Coagulation Factors; D006490:Hemostatics; D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; C065655:anti-inhibitor coagulant complex; D015942:Factor VIIa