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36th Hemophilia Symposium Hamburg 2005 by G Auerswald
About 31% of the patients with a factor VI replacement therapy develop a factor VI 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 VI-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 VI antibodies in healthy people. Well-known predisposing factors for inhibitor formation are genetic features of factor VI, 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]) Abbreviations: TCR - T cell-receptor; APC - antigen presenting cell 36 I.Wieland et al.| ISBN 13 | 9783540367147 |
|---|---|
| ISBN 10 | 3540367144 |
| Author | G Auerswald |
| Condition | New |
| Binding Type | Paperback |
| Publisher | Springer |
| Year published | 2006-11-02 |
| Number of pages | 319 |
| Cover note | Book picture is for illustrative purposes only, actual binding, cover or edition may vary. |
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