Primary hyperfibrinolysis: Facts and fancies

Fibrinolysis is a complex process that controls both hemostasis and thrombosis. The regulation of the fibrinolytic system is mediated through a wide array of cofactors and inhibitors that maintain the hemostatic balance in a delicate equilibrium. As a consequence, disturbances in the fibrinolysis pathway are associated with various disease states, ranging from thrombotic to hemorrhagic clinical phenotypes. In particular, a number of inherited and acquired disorders are associated with an enhanced fibrinolysis leading to a bleeding tendency that in some cases may be life-threatening. Hyperfibrinolysis has been classified into primary and secondary forms but such differentiation, which may have important treatment implications, is still controversial. This narrative review will be focused on inherited and acquired conditions associated with primary hyperfibrinolysis.