Review of immune tolerance induction in hemophilia A

Blood Reviews
S J SchepM L Boes

Abstract

At first sight the bleeding disorder hemophilia A seems to have little in common with immune disorders, but immunology research intersects with other disciplines including hematology. Nowadays, the most important complication in the treatment of hemophilia A is the development of neutralizing antibodies (inhibitors) against exogenous administered factor VIII (FVIII), which occurs in approximately 30% of all patients with severe hemophilia A. This antibody response renders FVIII replacement therapy ineffective, thereby increasing the risk for uncontrollable bleeding and morbidity, decreasing quality of life and increasing healthcare costs. The only proven effective therapy to eradicate these inhibitors is immune-based. Using a protocol called "immune tolerance induction" (ITI), the repeated and frequent administration of FVIII under non-inflammatory conditions downregulates the established antibody response and induces immune tolerance. There has been progress in research clarifying the mechanisms that mediate tolerance induction using ITI, both from patient studies and from research in cell culture and animal-based models. Peripheral tolerance induction to FVIII involves the apoptosis of antigen-specific B-memory cells, anergy ...Continue Reading

Citations

May 10, 2019·British Journal of Haematology·Guy Young
Jul 23, 2019·Haemophilia : the Official Journal of the World Federation of Hemophilia·Denise E SabatinoDonna M DiMichele
Jun 20, 2020·Haemophilia : the Official Journal of the World Federation of Hemophilia·Midori Shima, Robert F Sidonio
Mar 17, 2020·Frontiers in Immunology·Sadaf AtiqiGerrit J Wolbink
Aug 19, 2020·International Journal of Molecular Sciences·Sanne J van der VeenMirjam Langeveld
May 7, 2020·Frontiers in Immunology·Alessandra De Paula PohlDavid W Scott
Jan 31, 2020·Frontiers in Immunology·Sebastien Lacroix-DesmazesKathleen P Pratt
Nov 21, 2020·Haemophilia : the Official Journal of the World Federation of Hemophilia·Brian BoylanConnie H Miller
Apr 27, 2021·HemaSphere·Sarah J SchepMarianne Boes
May 18, 2021·Molecular Therapy. Nucleic Acids·Wen-Yi LinLiang Tang
Aug 21, 2020·Current Topics in Medicinal Chemistry·Simon N MbuguaMartin O Onani

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