Hemophilia A: the Use of Immune Tolerance Induction Therapy in the Treatment of the Inhibitory Form
Hemophilia A is an inherited bleeding disorder. It is characterized by a decrease or impaired synthesis of blood coagulation factors VIII. Hemophilia A is an orphan disease, due to the rare frequency of occurrence. One of the most serious problems in the treatment of patients with haemophilia is the synthesis of inhibitors that produce in response to the introduction of a genetically deficient coagulation factor, which occurs in approximately 30% of all patients with severe haemophilia A. The first line of therapy in patients with inhibitory form of haemophilia A is the inhibitor elimination, which is achieved by inducing immune tolerance. Usage of a concentrate of blood coagulation factors VIII and von Willebrand factor "Octanate", obtained from human plasma, in accordance with the Bonn protocol, made it possible to achieve a stable positive effect in most of these patients. "Treating algorithm for patients with an inhibitory form of haemophilia A" in the Republic of Belarus determines the range of pharmacotherapy possibilities, considering the characteristics of the course of the disease: blood coagulation factor VIII (immune tolerance induction); anti-inhibitor coagulant complex - as a prevention and relief of bleeding episodes. The method of immune tolerance induction, including monotherapy with Octanate, is clinically effective in the treatment of patients with inhibitory form of haemophilia A. This fact justifies the social and economic feasibility of using this therapy.