Abstract

As is known, fresh frozen donor plasma is a blood component, which includes thermolabile and thermostable coagulation factors. Indications for transfusion of fresh frozen plasma are conditions in which there is a deficiency of plasma coagulation factors — bleeding, overdose of indirect anticoagulants, disseminated intravascular coagulation syndrome in the hypocoagulation phase. However, there are conditions not caused by the deficiency of all clotting factors, but associated with a lack of any link. These conditions include the most common congenital hemophilia A (factor VIII deficiency) and hemophilia B associated with factor IX deficiency, von Willebrand disease, as well as rare coagulopathy caused by a deficiency of other coagulation factors (proaccelerin, proconvertin, factor XI, fibrinogen). In such situations, it is more appropriate to replace the missing blood factors with drugs obtained from donor plasma (plasma coagulation factors) or obtained by genetic engineering (recombinant coagulation factors). Recombinant coagulation factors (RCFs) are lyophilized preparations of glycoproteins that have the biological activity of a blood coagulation factor isolated from plasma and are intended for replacement therapy in diseases associated with disorders of the blood coagulation system. The use of recombinant coagulation factors, unlike plasma ones, completely eliminates the risk of transmission of bloodborne infections, and also makes it possible to establish production regardless of the presence or absence of donor plasma reserves. The development, production and use of recombinant coagulation factors is a completely new step in the development of hematology and the treatment of hereditary diseases, which include congenital coagulopathy.

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