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Quebec platelet disorder
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Quebec platelet disorder : ウィキペディア英語版
Quebec platelet disorder

Quebec Platelet Disorder (QPD) is a rare, autosomal dominant bleeding disorder described in a family from the province of Quebec in Canada.
==Pathophysiology==
The disorder is characterized by large amounts of the fibrinolytic enzyme urokinase-type plasminogen activator (u-PA) in platelets.〔Kahr, 2001〕 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.〔Sheth, 2003〕 These proteins include platelet factor V, Von Willebrand factor, fibrinogen, thrombospondin-1, and osteonectin.〔 There is also a quantitative deficiency in the platelet protein multimerin 1 (MMRN1). Furthermore, upon QPD platelet activation, u-PA can be released into forming clots and accelerate clot lysis, resulting in delayed-onset bleeding (12-24hrs after injury).〔Diamandis & Adam, 2006〕

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