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…mehr
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 ofinhibitors 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.
Epidemiology.- HIV Infection and Causes of Death in Patients with Hemophilia in Germany (Year 2004/2005 Survey).- Development of the German Hemophilia Register.- National Hemophilia Registry - Source of Information about the Quality of Hemophilia Medical Care in a Developing Country.- Hemophilia Therapy - Management of Bleedings and Inhibitors.- Regulation of Factor VIII Life-Cycle by Receptors from LDL Receptor Superfamily.- Update of the Inhibitor-Immunology-Study.- Therapy of Acquired Hemophilia - Immunoadsorption and Rituximab Treatment for Immunosuppression and Substitution of Coagulation Factors.- Orthopedic Treatment in Hemophiliacs.- Osteoporosis and Hemophilia - Is there a Correlation and Is there a Problem?.- Orthopedic Evaluation of the Lower Extremity in 249 Children: A Multicenter Trial.- Simultaneous Bilateral Total Knee Arthroplasty in Hemophilic Arthopathy.- Alternative Therapy such as the Acupuncture of the Skull for Hemophilic Arthropathy.- Endoprosthetics of Large Joints in Patients Infected with HIV.- Hemostaseologic Diagnostics.- Factor VIII Methods: Which Assay Principle for which Indication?.- Global ECAT Survey on Factor VIII Inhibitor Testing: Results of an Inter-Laboratory Pilot Study.- A Practical Concept for Pre-Operative Identification and Improved Management of Patients at Risk for Bleeding.- Pediatric Hemostaseology.- Intravascular Tissue Factor in Cord vs Adult Whole Blood.- Perioperative Coagulation Screening in Children - Reasons and Results.- Thrombin Generation in Children.- Free Lectures.- Working-Group of the German Hemophilia Assistants - History, Purpose and Goals.- The Endogenous Thrombin Potential as a New Parameter for the Peri-Operative Monitoring in Conjunction with Endo-Prosthetic Supply due to Hemophilic Arthropathy.- Identification of Inhibitor Epitopes in Acquired Hemophilia by Phage Display.- Spectrum of Molecular Defects and Mutation Detection Rate in Patients with Mild and Moderate Hemophilia A.- Recombinant Factor VIIa for Major Surgery in Severe Factor XI Deficiency: Pharmacodynamic Monitoring Using Thromboelastometry.- Poster.- Establishment of a Web-Based Documentation System for Quality Assurance of Hemophilia Treatment in Mecklenburg/Western Pomeranian: a Pilot Study.- Successful Angiographic Embolization of Recurrent Elbow Joint Bleeds in one Patient with Severe Hemophilia A.- Evaluation of Thrombotic Events in Hemophiliacs Undergoing Major Orthopedic Surgery Without Thrombosis Prophylaxis.- Motivating Patients and Parents to Document Treatment Correctly.- Fit for Life Competition: Everyone's a Winner.- Blood Borne Infections in Hemophiliacs in a Developing Country. A Single Center Experience.- Hemophilia Center Frankfurt - Twinning Center for Nairobi/Kenya.- Economic Evaluation of Orthopedic and Surgical Interventions in Hemophiliacs - Not Only Direct Medical Costs Matter.- The Occurrence of Factor VIII Inhibitor in a Patient with Mild Hemophilia A - During Treatment with Interferon for Chronic Hepatitis C.- Severe Hemophilia A Patient with High-Titer Inhibitor, use of TGA in the Monitoring of Bypassing Therapy.- EUREKA - an European Registry for Orthopedic Surgery in Hemophiliacs with Inhibitors.- Double Balloon Enteroscopy (DBE) with Argon Plasma Laser Coagulation (APC) for a Patient with Heyde's Syndrome.- Successful Liver Transplantation in a Patient with Anti-Thrombocyte Antibodies and Severe Hemophilia A.- Peri- and Postoperative Course of 95 Patients with von-Willebrand's Disease.- Pregnancy in a Patient with Congenital Antithrombin Deficiency.- Successful Inhibitor-Elimination with Rituximab in Acquired Hemophilia A and a Patient with a Carrier Status for Hemophilia A: Two Case Reports.- An Interesting Family Case of von-Willebrand-Syndrome.- Clinical Manifestations of Dysfibrinogenemia in Relation to the Fibrinogen Gene Mutation.- Thrombin Generation in a Hemophilic Newborn.- Rapid and Sensitive Detection of Heterozygous Deletions of one or more Exons in Hemophilia A Females by Multiplex PCR and DHPLC Technique.- Molecular Genetic Analysis in Patients with Inherited Factor V Deficiency.- Expression Analysis of C1-Inhibitor Mutants Confirms Causality of Missense Mutations for Hereditary Angioedema.- Expression of the ?-Glutamyl Carboxylase (GGCX) Containing the Arg485Pro Mutation Found in two Unrelated VKCFD1 Patients.- Concentration of Soluble Endothelial Protein C Receptor (EPCR) in Plasma in Relation to Age, Sex, BMI and Hemostasis Parameters.- Thrombin Generation is Age-Dependent in Children as well as in Adults.- Effects of PFA-100 in Preoperative Screening for von Willebrand Disease in 310 Patients.- Control of Aspirin Effect in Chronic Cardiovascular Patients Using two Whole Blood Platelet Function Assays: PFA-100 and Multiple Electrode Aggregometry.- Difficulties in the Interpretation of the Term »Patient-Related« in the Scope of the Introduction of a Remuneration of Additional Payments According to the OPS.- Factor VIII as Positive Regulator of Activated Platelets.- Characterization of Three Novel Mutations in the Sodium Binding Site of Coagulation Factor X.- Characterization of a Mutation in the 5? Flanking Region and a Novel IVS7 Splice site Mutation in a Patient with Severe FVII Deficiency.- On the Molecular Basis of Warfarin Resistance in Rats.- Influence of Factor VHR2 on Endogenous Thrombin Potential and Clinical Phenotype in Factor VII Deficiency.- Splice Site Mutations Effect on the F8 mRNA Splicing.- Species-Specific Variation of VKORC1-Activity and Resistance to Warfarin.- Various Missense Mutations in the Vitamin K Epoxide Reductase Complex Subunit 1 (VKORC1) Cause Hereditary Coumarin Resistance.- Establishment of an International Registry of Patients with Congenital FXIII Deficiency.- The Impact of Freezing of Plasma Samples, AB0 Blood Group and Acute-Phase Reaction on Detecting Mild Factor VIII Deficiency and Increased Factor VIII Levels as a Risk Factor for Venous Thromboembolism.- Thrombin Generation in Severely Obese Children.
Epidemiology.- HIV Infection and Causes of Death in Patients with Hemophilia in Germany (Year 2004/2005 Survey).- Development of the German Hemophilia Register.- National Hemophilia Registry - Source of Information about the Quality of Hemophilia Medical Care in a Developing Country.- Hemophilia Therapy - Management of Bleedings and Inhibitors.- Regulation of Factor VIII Life-Cycle by Receptors from LDL Receptor Superfamily.- Update of the Inhibitor-Immunology-Study.- Therapy of Acquired Hemophilia - Immunoadsorption and Rituximab Treatment for Immunosuppression and Substitution of Coagulation Factors.- Orthopedic Treatment in Hemophiliacs.- Osteoporosis and Hemophilia - Is there a Correlation and Is there a Problem?.- Orthopedic Evaluation of the Lower Extremity in 249 Children: A Multicenter Trial.- Simultaneous Bilateral Total Knee Arthroplasty in Hemophilic Arthopathy.- Alternative Therapy such as the Acupuncture of the Skull for Hemophilic Arthropathy.- Endoprosthetics of Large Joints in Patients Infected with HIV.- Hemostaseologic Diagnostics.- Factor VIII Methods: Which Assay Principle for which Indication?.- Global ECAT Survey on Factor VIII Inhibitor Testing: Results of an Inter-Laboratory Pilot Study.- A Practical Concept for Pre-Operative Identification and Improved Management of Patients at Risk for Bleeding.- Pediatric Hemostaseology.- Intravascular Tissue Factor in Cord vs Adult Whole Blood.- Perioperative Coagulation Screening in Children - Reasons and Results.- Thrombin Generation in Children.- Free Lectures.- Working-Group of the German Hemophilia Assistants - History, Purpose and Goals.- The Endogenous Thrombin Potential as a New Parameter for the Peri-Operative Monitoring in Conjunction with Endo-Prosthetic Supply due to Hemophilic Arthropathy.- Identification of Inhibitor Epitopes in Acquired Hemophilia by Phage Display.- Spectrum of Molecular Defects and Mutation Detection Rate in Patients with Mild and Moderate Hemophilia A.- Recombinant Factor VIIa for Major Surgery in Severe Factor XI Deficiency: Pharmacodynamic Monitoring Using Thromboelastometry.- Poster.- Establishment of a Web-Based Documentation System for Quality Assurance of Hemophilia Treatment in Mecklenburg/Western Pomeranian: a Pilot Study.- Successful Angiographic Embolization of Recurrent Elbow Joint Bleeds in one Patient with Severe Hemophilia A.- Evaluation of Thrombotic Events in Hemophiliacs Undergoing Major Orthopedic Surgery Without Thrombosis Prophylaxis.- Motivating Patients and Parents to Document Treatment Correctly.- Fit for Life Competition: Everyone's a Winner.- Blood Borne Infections in Hemophiliacs in a Developing Country. A Single Center Experience.- Hemophilia Center Frankfurt - Twinning Center for Nairobi/Kenya.- Economic Evaluation of Orthopedic and Surgical Interventions in Hemophiliacs - Not Only Direct Medical Costs Matter.- The Occurrence of Factor VIII Inhibitor in a Patient with Mild Hemophilia A - During Treatment with Interferon for Chronic Hepatitis C.- Severe Hemophilia A Patient with High-Titer Inhibitor, use of TGA in the Monitoring of Bypassing Therapy.- EUREKA - an European Registry for Orthopedic Surgery in Hemophiliacs with Inhibitors.- Double Balloon Enteroscopy (DBE) with Argon Plasma Laser Coagulation (APC) for a Patient with Heyde's Syndrome.- Successful Liver Transplantation in a Patient with Anti-Thrombocyte Antibodies and Severe Hemophilia A.- Peri- and Postoperative Course of 95 Patients with von-Willebrand's Disease.- Pregnancy in a Patient with Congenital Antithrombin Deficiency.- Successful Inhibitor-Elimination with Rituximab in Acquired Hemophilia A and a Patient with a Carrier Status for Hemophilia A: Two Case Reports.- An Interesting Family Case of von-Willebrand-Syndrome.- Clinical Manifestations of Dysfibrinogenemia in Relation to the Fibrinogen Gene Mutation.- Thrombin Generation in a Hemophilic Newborn.- Rapid and Sensitive Detection of Heterozygous Deletions of one or more Exons in Hemophilia A Females by Multiplex PCR and DHPLC Technique.- Molecular Genetic Analysis in Patients with Inherited Factor V Deficiency.- Expression Analysis of C1-Inhibitor Mutants Confirms Causality of Missense Mutations for Hereditary Angioedema.- Expression of the ?-Glutamyl Carboxylase (GGCX) Containing the Arg485Pro Mutation Found in two Unrelated VKCFD1 Patients.- Concentration of Soluble Endothelial Protein C Receptor (EPCR) in Plasma in Relation to Age, Sex, BMI and Hemostasis Parameters.- Thrombin Generation is Age-Dependent in Children as well as in Adults.- Effects of PFA-100 in Preoperative Screening for von Willebrand Disease in 310 Patients.- Control of Aspirin Effect in Chronic Cardiovascular Patients Using two Whole Blood Platelet Function Assays: PFA-100 and Multiple Electrode Aggregometry.- Difficulties in the Interpretation of the Term »Patient-Related« in the Scope of the Introduction of a Remuneration of Additional Payments According to the OPS.- Factor VIII as Positive Regulator of Activated Platelets.- Characterization of Three Novel Mutations in the Sodium Binding Site of Coagulation Factor X.- Characterization of a Mutation in the 5? Flanking Region and a Novel IVS7 Splice site Mutation in a Patient with Severe FVII Deficiency.- On the Molecular Basis of Warfarin Resistance in Rats.- Influence of Factor VHR2 on Endogenous Thrombin Potential and Clinical Phenotype in Factor VII Deficiency.- Splice Site Mutations Effect on the F8 mRNA Splicing.- Species-Specific Variation of VKORC1-Activity and Resistance to Warfarin.- Various Missense Mutations in the Vitamin K Epoxide Reductase Complex Subunit 1 (VKORC1) Cause Hereditary Coumarin Resistance.- Establishment of an International Registry of Patients with Congenital FXIII Deficiency.- The Impact of Freezing of Plasma Samples, AB0 Blood Group and Acute-Phase Reaction on Detecting Mild Factor VIII Deficiency and Increased Factor VIII Levels as a Risk Factor for Venous Thromboembolism.- Thrombin Generation in Severely Obese Children.
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