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Opis

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]) Abbreviations: TCR T cell-receptor; APC antigen presenting cell 36 I.Wieland et al.

Szczegóły książki

Kategoria Książki po angielsku Medicine Clinical & internal medicine Haematology

• Pełny tytuł: 36th Hemophilia Symposium Hamburg 2005
• Podtytuł: Epidemiology; Hemophilia Therapy - Management of Bleedings and Inhibitors; Orthopedic Treatment in Hemophiliacs; Hemostaseologic Diagnosis; Pediatric Hemostaseology; Free Lectures
• Autor: Inge Scharrer, Wolfgang Schramm
• Język: Angielski
• Oprawa: Miękka
• Liczba stron: 319
• EAN: 9783540367147
• ISBN: 3540367144
• ID: 01562389
• Wydawca: Springer-Verlag Berlin and Heidelberg GmbH & Co. KG
• Waga: 545 g
• Wymiary: 235 × 155 × 20 mm
• Data wydania: 02. November 2006

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