The plasminogen-plasmin system involves proteolytic enzymes which are primarily responsible for the degradation of fibrin deposits in blood vessels. Through intricate interactions between the various components and inhibitors, a balance is maintained between profibrinolysis and impaired fibrinolytic activity. Several hereditary defects have been described affecting functional plasminogen concentrations, plasminogen activator levels, and plasminogen activator inhibitor activity. These defects have been implicated as risk factors for thrombosis based on a multitude of case reports associating impaired fibrinolysis with thrombosis. However, under close scrutiny, the role of decreased fibrinolysis as an etiologic factor in thrombosis has not been firmly established. Rather, dysfibrinolysis may manifest itself through an accentuation of an underlying thrombophilic state such as recurrent thrombotic episodes. Further evaluation of impaired fibrinolytic activity in conjunction with an underlying thrombophilic condition is warranted.
Historically, the mortality rate of thrombotic thrombocytopenic purpura (TTP) approached 100%. However, by the 1980's, new therapy was instituted with a vast improvement in survival to 90%. The exact pathogenesis of TTP remains elusive. Yet, despite incomplete understanding of the pathophysiology, outcome has improved due to increased awareness of the symptomatology leading to earlier diagnosis and better supportive care, in addition to effective therapy with plasma exchange. TTP represents a disease in which prompt diagnosis and treatment can lead to a critical difference in clinical outcome.
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