Abstract:
Rare coagulation disorders (RCD) include monogenic coagulopathies caused by a deficiency of plasma proteins involved in hemostasis, not related to von Willebrand disease and hemophilia A or B. RBCD include hereditary deficiencies or abnormalities of fibrinogen, prothrombin (factor II), blood coagulation factors V, VII, X, XI, XII, XIII. All these violations in the vast majority of cases lead to violations of the formation of fibrin. Blood clotting is the process by which normal liquid blood turns into a clot that closes the damaged vessel and protects against bleeding. The external and internal pathway of thrombus formation proceeds with the participation of coagulation factors. This process involves the formation of fibrin, the protein that forms the basis of a blood clot. Fibrin as a scaffold gives the thrombus strength. With coagulopathy, due to a lack of coagulation factors, fibrin formation does not occur. Without fibrin, a thrombus is not able to securely attach to the site of damage. Uncontrolled bleeding occurs, which can lead to death. Hypofibrinogenemia is a pathological condition characterized by a decrease in the
content of fibrinogen in the blood below 2 g / l. Fibrinogen is the main protein of the
hemostasis system; a thrombus is formed from it, which stops bleeding when the vascular
wall is damaged.
