Prevention of inhibitor development in hemophilia A in 2016. A glimpse into the future?
Thanks to considerable progresses made over the last 30years, hemophilia benefits from the most efficacious and safe treatment among the many monogenic inherited disorders. The most challenging complication of replacement therapy in hemophilia A is the occurrence of alloantibodies against infused factor VIII (FVIII), thus predisposing the patients to increased morbidity and disability. Extensive research in this field has definitively unraveled that development of inhibitors in hemophilia A is a complex and multifactorial process, in which inherited and environmental factors dynamically interact. This narrative review, after providing a concise overview about the main genetic and non-genetic risk factors, is aimed to focus on prediction risk models and preventive strategies for minimizing the risk of developing inhibitors in hemophilia A patients.
Department of Hematology and Transfusion Medicine, Carlo Poma Hospital, Mantua, Italy. Electronic address: massimo.franchini@asst-mantova.it.
Massimo
M
Franchini
Section of Clinical Biochemistry, University of Verona, Verona, Italy.
Giuseppe
G
Lippi
D000818:Animals; D005169:Factor VIII / Q000235:genetics / Q000276:immunology* / Q000627:therapeutic use*; D006467:Hemophilia A / Q000235:genetics / Q000276:immunology* / Q000517:prevention & control / Q000628:therapy*; D006801:Humans; D007518:Isoantibodies / Q000276:immunology*; D009154:Mutation; D011994:Recombinant Proteins / Q000235:genetics / Q000276:immunology / Q000627:therapeutic use
D007518:Isoantibodies; D011994:Recombinant Proteins; C078147:F8 protein, human; D005169:Factor VIII
Alloantibody hemophilia A; Inhibitor; Prevention