Factor XIII (fibrin-stabilizing factor)

The reference value (norm) of factor XIII activity in blood plasma is 100%.

Factor XIII (fibrin-stabilizing factor, fibrinase) is a β ₂ -glycoprotein. It is present in the vascular wall, platelets, erythrocytes, kidneys, lungs, muscles, and placenta. In plasma, it is found as a proenzyme associated with fibrinogen.

Factor XIII under the influence of thrombin is converted into the active form XIIIa, which, when forming a fibrin clot, ensures the formation of cross-linked forms of fibrin. Thrombi formed in the presence of fibrinase are very slowly lysed. With a decrease in the activity of factor XIII, clots disintegrate very quickly, even if the fibrinolytic activity of the blood is normal. When the wall of a blood vessel is damaged, factor XIII participates in the process of aggregation and adhesion of blood platelets. It has been established that a decrease in fibrinase activity is accompanied by a decrease in the adhesiveness and aggregation of platelets, and with an increase in fibrinase activity, these properties of platelets, on the contrary, increase.

Factor XIII characterizes phase III of blood coagulation: a decrease or increase in fibrinase activity is considered a factor of hemorrhagic or thrombotic risk.

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Congenital factor XIII deficiency

It is inherited in an autosomal recessive manner, mainly by men. The first clinical sign of fibrinase deficiency in 80% of patients is prolonged (for days, sometimes weeks) bleeding from the umbilical wound. Petechial bleeding is typical. Hemorrhages in the brain are possible. Slow wound healing is noted, postoperative hernias often form, and fractures heal poorly. All parameters in the coagulogram, except for a decrease in the concentration of factor XIII in the blood plasma, remain within normal limits.

In this disease, blood clotting (according to the coagulogram) is normal, but the fibrin clot is structurally unstable due to a deficiency of factor XIII. First of all, bleeding from the umbilical wound after the umbilical cord remnant falls off or is excised attracts attention, and the bleeding can be moderate, but necessarily prolonged, for 2-5 weeks, which is why factor XIII deficiency is also called "bleeding navel". In addition, prolonged gastrointestinal and intracranial bleeding is possible.

The diagnosis is confirmed by determining the content of factor XIII (significant decrease). Other coagulogram parameters, as well as the number of platelets, are unchanged.

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Treatment

Treatment is based on replacement therapy with blood coagulation factor VIII preparations: antihemophilic plasma at a rate of 10-20 ml/kg intravenously by drip or cryoprecipitate (1 dose contains 75 U of factor XIII) at a rate of 0.3 dose/kg of the child’s body weight intravenously.

Acquired factor XIII deficiency

It is detected in patients with vitamin C deficiency, radiation sickness, leukemia, cirrhosis, hepatitis, cancer with liver metastases, lymphoma, with DIC syndromes, in those who have undergone adrenalectomy, after taking indirect anticoagulants. A decrease in factor XIII in the blood in these diseases is due to a violation of its synthesis or its consumption during the DIC syndrome.

In case of long-term and poorly healing wounds and fractures, it is recommended to conduct a study of the activity of factor XIII, since in some cases such phenomena may be associated with its deficiency (factor XIII stimulates the development of fibroblasts).

The minimum hemostatic level of factor XIII activity in the blood to stop bleeding is 1-2%; at a lower content, stopping bleeding without administering factor XIII to the patient is impossible.

In patients with thromboembolic complications, atherosclerosis, after surgery, childbirth, after the administration of adrenaline, glucocorticosteroids, pituitrin, fibrinase activity is often increased.

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