Heterogeneity of congenital afibrinogenemia, from epidemiology to clinical consequences and management

Fibrinogen is a complex protein playing major role in coagulation. Congenital afibrinogenemia, characterized by the complete absence of fibrinogen, associated with hemostatic defects. Even though clinical course unpredictable and can be completely different among patients, severe bleeding prominent symptom. Patients are also at increased risk thrombosis sometimes suffer from spontaneous spleen rupture, bone cysts defective wound healing. Due to relative rarity there no evidence-based strategies for helping physicians care these patients. supplementation keystone prevent or treat events. In addition, pleiotropic numerous physiological roles immunity, angiogenesis tissue repair, involved many diseases. Indeed, depletion fibrinogen animal models infections, tumors neurological diseases has an effect on course. The consequences patients afibrinogenemia still need investigated.