afibrinogenemia

A B B D E F G And K L M N O U R C T Y P X C H W E I

Afibrinogenemia - a hereditary disease caused by lack of blood plasma fibrinogen.

When afibrinogenemia blood clotting does not occur. This occurs when the plasma content of less than 0.5 g / L (50 mg / 100 ml) of fibrinogen; normal fibrinogen content amounts to 2-4 g / L (200-400 mg / 100 ml).

Afibrinogenemia extremely rare, inherited in an autosomal recessive manner. In this case, any injury in the child leads to bleeding, and when the child begins to walk, there ecchymosis, hematoma, bleeding in the joint cavity. Children with afibrinogenemia rarely live to adulthood. Diagnosis is incoagulability when detecting absence of blood and fibrinogen in the blood plasma with normal platelet count.

In clinical practice often occurs gipofibrinogenemia - reduced levels of fibrinogen in blood plasma. Gipofibrinogenemia usually acquired as a result of lack of education in fibrinogen with liver disease, and increased its dissolution - fibrinolysis. When hypofibrinogenaemia slowed blood clotting, formed a loose clot or friability. Gipofibrinogenemia manifested ecchymosis, hematoma formation at the slightest trauma, hemorrhage (nasal, uterine, etc.). Blood clotting begins, but after a few minutes or hours, the blood becomes liquid again. This pattern is observed in liver cirrhosis and acute necrosis, a number of malignant tumors with metastases in the bone marrow, acute leukemia, shock of any origin, as well as the complicated birth (fetal death, premature detachment of the placenta or manual removal). The cause of acute hypofibrinogenaemia accompanied by uterine bleeding, can be intravascular coagulation with power consumption of fibrinogen under the influence of penetration into the blood from the placenta thromboplastin and amniotic fluid.

The main treatment is hypofibrinogenaemia afibrinogenemia and transfusion of plasma and fibrinogen.