This book contains the contribution to the 37th Hemophilia Symposium, Hamburg 2006. The main topics are epidemiolgy, treatment of inhibitors in hemophiliacs, hemophilic arthropathy and synovitis, relevant hemophilia treatment 2006, and pediatric hemostasiology. The volume is rounded off by numerous free papers and posters on hemophilia, casuistics, and diagnostics.
About 31% of the patients with a factor VIII replacement therapy develop a factor VIII inhibitor.From these are 23% low-responder ( 5BE) [8].In the case of severe hemophilia B, about 10.5% of the patients develop inhibitory antibodies [9]. Anti-factor VIII-antibodies are also seen in 15-78% healthy people without hemophilia [7, 17, 19]. Lacroix-Desmazes et al. [10, 11] showed anti-idiotypic antibodies neutralizing the inhibitory activity of the an- factor VIII antibodies in healthy people. Well-known predisposing factors for inhibitor formation are genetic features of factor VIII, which include large deletions, nonsense mutations or intrachromosomal recombinations [5, 23].Also, ethnic groups other than Caucasians (e.g.Africans) have a higher risk of developing inhibitors.Other risk factors are presumably de- ved from the immune system. For instance, a reduction of the inhibitor was seen with lower CD4+ T helper cell counts in HIV positive hemophilic patients [3,4].The development of inhibitors is very likely to be a Th-2 mediated event where cyto- nes and their receptors, T-cell receptors and the Major Histocompatibility Complex may also play an important role. Theoretical Background The substituted factor is an unknown protein for patients with a severe he- philia. Fig. 1. The normal immunoresponse (according to BAENKLER [2]) TCR - T cell-receptor; APC - antigen presenting cell 36 I.Wieland et al.
This book contains the contribution to the 35th Hemophilia Symposium, Hamburg 2004. The main topics are epidemiology, risk of infections and inhibitors in hemophilia, chronic hemophilic synovitis and long-term results of orthopedic treatment, laboratory diagnostics and pediatric hemostaseology. The volume is rounded off by numerous free papers and posters on hemophilia and hemorrhagic disorders and inhibitors in hemophilia.
This book contains the contributions to the 34th Hemophilia Symposium, Hamburg 2003. The main topics are HIV infection and epidemiology, management of bleedings in hemophiliacs with inhibitors, orthopedic problems and therapy in hemophiliacs, therapy with protein C and pediatric hemostaseology. The volume is rounded off by numerous free papers and posters on hemophilia, hemophiliacs with inhibitors, thrombophilia and molecular diagnostics.
This book contains the contributions to the 33rd Hemophilia Symposium, Hamburg 2002. The main topics are epidemiology, new findings and possibilities in the therapy of antibodies; therapeutic exercise and sport. The volume is rounded off by numerous free papers and posters on hemophilia and hemorrhagic disorders, thrombophilic disorders and associated topics.
Evaluation of the results revealed that the majority of participants prefer treat ment in a hemophilia treatment center with a high reputation, whereas only 2. 3% re quested standardized treatment regimens for all treatment centers, only about 20% would change to a nearer center.
This book contains the contributions to the 30th Hemophilia Symposium, 1999. The main topics are HIV infection, inhibitors in hemophilia, modern treatment of hemophilia, drug-induced thrombophilia and pediatric hemostasiology. The volume is rounded off by numerous free papers and posters on hemophilia and associated topics.