Anomalies constitutionnelles de la fibrinolyse et syndromes hémorragiques

Fibrinolysis is known as the plasma and cellular mechanisms leading to destruction of the blood clot, but it is also involved in tissue development. Its dysregulation can contribute to the occurrence of thrombosis and bleeding. For bleeding, two inhibitor deficiencies have been identified as clinically relevant, involving α2-antiplasmin and PAI-1. Although variable, the bleeding symptoms are similar, as are treatment options. Both deficiencies are characterized by a very low occurrence rate, are not detected by current hemostasis evaluation and therefore require specific measurements which are not always performed. They need to be sought at least in well documented cases of persistent hemorrhagic profile once more frequent biological causes have been set aside like von Willebrand disease, a coagulation factor deficiency, thrombocytopathy. Once this diagnosis has been performed, the treatment options are simple, and evaluation of the relatives is needed.