THE USE OF EMICIZUMAB IN A CHILD WITH HEMOPHILIA A INHIBITORY FOREMA
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Abstract
Hemophilia is a disease caused by hereditary deficiency of plasma coagulation factors VIII (FVIII) (hemophilia A) or IX (FIX) (hemophilia B) and characterized by hematomic bleeding.
The main therapeutic measures in patients with hemophilia are aimed at replacing the deficient factor both during hemorrhagic manifestations and for preventive purposes. Due to frequent transfusions of blood products, approximately 30 % of patients with hemophilia A develop antibodies to factor VIII. Antibodies block the procoagulant activity of the injected factors VIII and IX, and this may cause the ineffectiveness of hemostatic therapy in the usually accepted volumes.
In October 2018, a new drug Emicizumab was registered in the territory of the Russian Federation, which is a bispecific humanized monoclonal antibodies based on immunoglobulin G4, which bind activated factor IX with factor X to replenish the function of the missing activated factor VIII. The article presents the first case in Kuzbass of using Emizizumab in a child with an inhibitory form of hemophilia A.
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References
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