BIOMAT Database Logo BIOMAT Database Logo

Reduction of Factor VIII Inhibitor Titers During Immune Tolerance Induction With Recombinant Factor VIII-Fc Fusion Protein. 2016

Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Department of Pediatrics, Uniformed Services University of the Health Sciences, Bethesda, Maryland.

The development of inhibitors toward factor VIII (FVIII) is a common and serious complication of hemophilia A (HA) therapy. Patients with hemophilia who develop inhibitors often undergo time- and resource-intensive immune tolerance induction (ITI) protocols. We report a 15-month-old male with severe HA and a high-titer inhibitor that occurred while receiving prophylactic treatment with recombinant FVIII (rFVIII), in whom significant inhibitor titer reduction was achieved with thrice weekly infusions of a new, prolonged half-life rFVIII-Fc fusion protein product (trade name Eloctate). Further studies are warranted to explore the potential of Eloctate in ITI protocols.

UI MeSH Term Description Entries
D007108 Immune Tolerance The specific failure of a normally responsive individual to make an immune response to a known antigen. It results from previous contact with the antigen by an immunologically immature individual (fetus or neonate) or by an adult exposed to extreme high-dose or low-dose antigen, or by exposure to radiation, antimetabolites, antilymphocytic serum, etc. Immunosuppression (Physiology),Immunosuppressions (Physiology),Tolerance, Immune
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D011961 Receptors, Fc Molecules found on the surface of some, but not all, B-lymphocytes, T-lymphocytes, and macrophages, which recognize and combine with the Fc (crystallizable) portion of immunoglobulin molecules. Fc Receptors,Fc Receptor,Receptor, Fc
D011993 Recombinant Fusion Proteins Recombinant proteins produced by the GENETIC TRANSLATION of fused genes formed by the combination of NUCLEIC ACID REGULATORY SEQUENCES of one or more genes with the protein coding sequences of one or more genes. Fusion Proteins, Recombinant,Recombinant Chimeric Protein,Recombinant Fusion Protein,Recombinant Hybrid Protein,Chimeric Proteins, Recombinant,Hybrid Proteins, Recombinant,Recombinant Chimeric Proteins,Recombinant Hybrid Proteins,Chimeric Protein, Recombinant,Fusion Protein, Recombinant,Hybrid Protein, Recombinant,Protein, Recombinant Chimeric,Protein, Recombinant Fusion,Protein, Recombinant Hybrid,Proteins, Recombinant Chimeric,Proteins, Recombinant Fusion,Proteins, Recombinant Hybrid
D003888 Desensitization, Immunologic Immunosuppression by the administration of increasing doses of antigen. Though the exact mechanism is not clear, the therapy results in an increase in serum levels of allergen-specific IMMUNOGLOBULIN G, suppression of specific IgE, and an increase in suppressor T-cell activity. Allergen Immunotherapy,Allergy Shots,Hyposensitization Therapy,Immunotherapy, Allergen,Venom Immunotherapy,Immunologic Desensitization,Therapy, Hyposensitization,Allergen Immunotherapies,Allergy Shot,Desensitizations, Immunologic,Hyposensitization Therapies,Immunologic Desensitizations,Immunotherapy, Venom,Shot, Allergy,Venom Immunotherapies
D005169 Factor VIII Factor VIII of blood coagulation. Antihemophilic factor that is part of the factor VIII/von Willebrand factor complex. Factor VIII is produced in the liver and acts in the intrinsic pathway of blood coagulation. It serves as a cofactor in factor X activation and this action is markedly enhanced by small amounts of thrombin. Coagulation Factor VIII,Factor VIII Clotting Antigen,Factor VIII Coagulant Antigen,Factor VIII Procoagulant Activity,Thromboplastinogen,Blood Coagulation Factor VIII,F VIII-C,Factor 8,Factor 8 C,Factor Eight,Factor VIIIC,Hyate-C,Hyatt-C,F VIII C,Hyate C,HyateC,Hyatt C,HyattC
D006467 Hemophilia A The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage. Factor VIII Deficiency,Hemophilia,Autosomal Hemophilia A,Classic Hemophilia,Deficiency, Factor VIII,Factor 8 Deficiency, Congenital,Factor VIII Deficiency, Congenital,Haemophilia,Hemophilia A, Congenital,Hemophilia, Classic,As, Autosomal Hemophilia,Autosomal Hemophilia As,Classic Hemophilias,Congenital Hemophilia A,Congenital Hemophilia As,Hemophilia A, Autosomal,Hemophilia As,Hemophilia As, Autosomal,Hemophilia As, Congenital,Hemophilias, Classic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D019774 Blood Coagulation Factor Inhibitors Substances, usually endogenous, that act as inhibitors of blood coagulation. They may affect one or multiple enzymes throughout the process. As a group, they also inhibit enzymes involved in processes other than blood coagulation, such as those from the complement system, fibrinolytic enzyme system, blood cells, and bacteria. Blood Coagulation Factor Inhibitor,Inhibitor, Blood Coagulation Factor,Inhibitor, Coagulation Factor, Blood,Inhibitors, Blood Coagulation Factor,Inhibitors, Coagulation Factor, Blood

Related Publications

Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Recombinant factor VIII Fc fusion protein for first-time immune tolerance induction: final results of the verITI-8 study.
April 2023, Blood,
Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Recombinant factor VIII Fc fusion protein for immune tolerance induction in patients with severe haemophilia A with inhibitors-A retrospective analysis.
March 2018, Haemophilia : the official journal of the World Federation of Hemophilia,
Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Immune tolerance induction using Fc-fusion-protein recombinant factor IX in severe haemophilia B.
November 2021, Haemophilia : the official journal of the World Federation of Hemophilia,
Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Changing recombinant factor VIII to plasma-derived factor VIII during immune tolerance induction.
January 2024, Pediatric hematology and oncology,
Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Population pharmacokinetics of recombinant factor VIII Fc fusion protein.
January 2015, Clinical pharmacology in drug development,
Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Recombinant factor VIII Fc fusion protein drives regulatory macrophage polarization.
November 2018, Blood advances,
Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Indirect comparisons of efficacy and weekly factor consumption during continuous prophylaxis with recombinant factor VIII Fc fusion protein and conventional recombinant factor VIII products.
May 2017, Haemophilia : the official journal of the World Federation of Hemophilia,
Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Real-world data of immune tolerance induction using recombinant factor VIII Fc fusion protein in patients with severe haemophilia A with inhibitors at high risk for immune tolerance induction failure: A follow-up retrospective analysis.
January 2021, Haemophilia : the official journal of the World Federation of Hemophilia,
Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Recombinant factor VIII Fc (rFVIIIFc) fusion protein reduces immunogenicity and induces tolerance in hemophilia A mice.
March 2016, Cellular immunology,
Charles L Groomes, and David M Gianferante, and Gary D Crouch, and Dina S Parekh, and David W Scott, and Kenneth Lieuw
Factor VIII moiety of recombinant Factor VIII Fc fusion protein impacts Fc effector function and CD16+ NK cell activation.
January 2024, Frontiers in immunology,
Copied contents to your clipboard!