36th Hemophilia Symposium Hamburg 2005: Epidemiology; Hemophilia Therapy - Management of Bleedings and Inhibitors; Orthopedic Treatment in Hemophiliacs; Hemostaseologic Diagnosis; Pediatric Hemostaseology; Free Lectures
Inge Scharrer, Wolfgang Schramm
Springer Science & Business Media, Feb 16, 2007 - Medical - 319 pages
About 31% of the patients with a factor VIII replacement therapy develop a factor VIII inhibitor.From these are 23% low-responder ( 5BE) and 77% high-responder ( 5BE) .In the case of severe hemophilia B,about 10.5% of the patients develop inhibitory antibodies . 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 ) Abbreviations: TCR – T cell-receptor; APC – antigen presenting cell 36 I.Wieland et al.
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36th Hemophilia Symposium acid adult WB analysis antibodies anticoagulant antigen antithrombin aPTT assay binding blood cells clinical clotting deficiency detected disorders endogenous thrombin potential epitopes exon factor VII deficiency factor VIII factor VIII inhibitor factor X fibrinogen Frau Dr FVHR2 FVII FVIII FVIII activity FVIII levels FVIII:Ag FVIII:C FVLeiden gene hemophilia center Hemophilia Symposium Hamburg hemophilic hemostasis hemostatic identified infection Klinikum knee laboratory liver measured Medizin Verlag Heidelberg Methods mutation neonatal normal obese orthopedic osteoporosis patients with hemophilia peptide phage plasma platelet platelet function protein prothrombin receptor recombinant residual rFVIIa Rituximab samples Scharrer/W Schramm sEPCR plasma sequence severe hemophilia showed splice Springer Medizin Verlag substitution surgery surgical Symposium Hamburg 2005 TFPI therapy Thromb Haemost thrombin thrombosis thrombotic transfusion treatment Universitätsklinik Verlag Heidelberg 2007 VIII concentrates vitamin K VKORC1 von Willebrand disease von Willebrand factor warfarin