The disorder is characterized by large amounts of the fibrinolytic enzyme urokinase-type plasminogen activator (u-PA) in platelets [3]. Consequently, stored platelet plasminogen is converted to plasmin, which is thought to play a role in degrading a number of proteins stored in platelet α-granules [4].
Quebec platelet disorder is listed as a \"rare disease\" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Quebec platelet disorder, or a subtype of Quebec platelet disorder, affects less than 200,000 people in the US population.