Abstract :
Hemophilia A (HA) is a X-linked recessive bleeding disorder occurring due to the deficiency of factor VIII (FVIII). It is treated by transfusion of plasma-derived (pdFVIII) or recombinant FVIII (rFVIII) concentrates. In ~25% of patients with severe Hemophilia A (SHA) inhibitory antibodies may get produced against FVIII, causing shortening of the FVIII half-life and consequently in nullification of its function. These antibodies are known as inhibitors. Bleeding episodes now become refractory to the standard treatment, making alternative therapeutic approaches like costly inhibitor bypassing agents necessary; consequently, increasing the morbidity and shrinking the quality of life of patients with Hemophilia A (PwHA).This review aimed to: (i) summarize the current knowledge on inhibitors in Hemophilia A and, (ii) enumerate the clinico-pathological variables related to inhibitor development.
Keyword :
Inhibitors, Hemophilia, Hemophilia A, Factor VIII, Neutralizing antibodies, Risk factors, Prevalence.