afibrinogenemia

Afibrinogenemia is a rare inherited blood disorder characterized by a significant reduction or absence of fibrinogen in the blood. Fibrinogen is a soluble protein produced by the liver and found in blood plasma, essential for blood clotting.12

When tissue damage occurs, fibrinogen is converted into fibrin, an insoluble protein that forms long fibrous chains. These chains entangle platelets, creating a mass that hardens into a blood clot, stopping bleeding. In individuals with afibrinogenemia, this conversion is impaired because there is insufficient fibrinogen. Consequently, blood clots cannot form properly, leading to difficulties in stopping bleeding. This condition is distinct from disseminated intravascular coagulation, although it can be a component of that syndrome.21