Insights into Disturbed Hemostasis in the Quebec Platelet Disorder Using Thromboelastography, Blood Clots Formed at Low Shear, and Perfusion Studies of Fibrinolysis.

Abstract The Quebec Platelet Disorder (QPD) is an inherited disorder associated with delayed bleeding, increased expression and storage of active urokinase-type plasminogen activator (u-PA) in platelets, normal to increased u-PA in plasma, consumption of platelet plasminogen activator inhibitor-1 (PAI-1), and increased plasmin generation in platelets with proteolysis of stored α-granule proteins, including factor V. Although …