HEREDITARY DEFICIENCY OF BLOOD COAGULATION FACTOR X, CLINICAL OBSERVATIONS. MEDICAL CENTER OF HEMATOLOGY OF THE MINISTRY OF HEALTH OF THE REPUBLIC OF UZBEKISTAN.

Blood coagulation factor X (Stewart Factor - Prower) - gamma globulin protein, proenzyme (protease). Factor Xa is a component of the prothrombin activator. This factor is produced in the liver. Vitamin K is required for its synthesis. Under the influence of several clotting factors (III, VII, VIII, IX), it passes into the active form, which has the designation Xa. It, in turn, together with the coagulation factor V and Ca2+ ions, forms an enzyme complex that acts as an activator of prothrombin. FX activation occurs in the initiating phase of blood coagulation with the participation of the TF-FVIIa complex and in the amplification phase with the tenase complex. Activated FX (FXa) and its cofactor, coagulation factor V (FV), are part of the prothrombinase complex. Coagulation factor X (FX) deficiency is a disorder in the plasma link of hemostasis with an autosomal recessive type of inheritance, characterized by a decrease in FX activity due to quantitative or qualitative defects in the FX protein. The prevalence of the disease is 1 patient: 1,000,000 population. FX deficiency is caused by various structural abnormalities in the F10 gene encoding FX.

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