Abstract
Fibrinolysis is a complex enzymatic process aimed at dissolving blood clots to prevent vascular occlusions. The fibrinolytic system is composed of a number of cofactors that, by regulating fibrin degradation, maintain the hemostatic balance. A dysregulation of fibrinolysis is associated with various pathological processes that result, depending on the type of abnormality, in prothrombotic or hemorrhagic states. This narrative review is focused on the congenital and acquired disorders of primary fibrinolysis in both adults and children characterized by a hyperfibrinolytic state with a bleeding phenotype.
Highlights
We summarize the main pathogenetic, laboratory, clinical and therapeutic characteristics of inherited and acquired bleeding conditions associated with primary hyperfibrinolysis, which includes qualitative or quantitative abnormalities of proteins involved in the fibrinolytic process
Primary hyperfibrinolysis is nowadays recognized as a distinct clinical bleeding entity associated with a variety of inherited and acquired disorders
Inherited bleeding disorders characterized by primary hyperfibrinolysis are a group of rare heterogeneous diseases, and their identification represents a diagnostic challenge that requires, besides a high-level clinical knowledge by physicians of these neglected diseases, the presence of specialized second-level hemostasis laboratories
Summary
Conditions causing a hypo- or hyper-fibrinolytic state are inherited, typically caused by a single molecular defect or more frequently acquired [6,7]. In this narrative review, we summarize the main pathogenetic, laboratory, clinical and therapeutic characteristics of inherited and acquired bleeding conditions associated with primary hyperfibrinolysis, which includes qualitative or quantitative abnormalities of proteins involved in the fibrinolytic process (see Table 1). XIII, t-PA, tissue plasminogen activator; u-PA, urokinase plasminogen activator; α2AP, α2 -antiplasmin; PAI-1, plasminogen activator inhibitor 1; TAFI, thrombin-activatable fibrinolysis inhibitor; FDP, fibrin degradation products.
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
