Inherited disorders of the fibrinolytic pathway

Deficiencies or excessive activation of the  fibrinolytic system can result in severe, lifelong bleeding disorders. The most severe clinical phenotype is caused by α2−Antiplasmin (α2−AP) deficiency which results in excess fibrinolysis due to the inability to inhibit plasmin. Another bleeding disorder due to a defect in the fibrinolyti c pathway results from Plasminogen activator inhibitor-1 (PAI-1) deficiency causing enhanced fibrinolysis due to the decreased inhibition of plasminogen activators resulting in increased conversion of plasminogen to plasmin.

Source: Transfusion and Apheresis Science - August 7, 2019 Category: Hematology Authors: Shilpa Jain, Suchitra S. Acharya Source Type: research