释义 |
activated protein C resistance
Activated Protein C Resistance An inherited (autosomal dominant) or acquired defect in the anticoagulant response to activated protein C, resulting in an increased risk of thrombosis. Clinical findings Increased risk of deep vein thrombosis (DVT), venous thrombosis; it is responsible for 20% to 50% of DVT. Pathogenesis Protein C, a key regulator of coagulation, circulates in an inactivated form and is activated by the binding of thrombin to thrombomodulin receptors on vascular endothelial cells; once activated, protein C lyses coagulation factors Va and VIIIa. Activated protein C resistance is caused by a failure of protein C to cleave Factor Va and/or Factor VIIIa.activated protein C resistance APC resistance Hematology A condition caused by an inherited defect in the anticoagulant response to APC and clinically characterized by ↑ venous thrombosis; it is responsible for 20-50% of DVT Pathogenesis Protein C, a key regulator of coagulation, circulates in an inactivated form and is activated by the binding of thrombin to thrombomodulin receptors on vascular endothelial cells; once activated, protein C lyses coagulation factors Va and VIIIa; APCR may be due to a selective defect in factor V coagulant functionactivated protein C resistance An abnormality in the regulation of blood clotting often found in patients with an unexplained THROMBOSIS. It is associated with a mutation in the gene for the clotting factor, Factor V, and may provide an explanation of the common genetic predisposition to thrombosis (thrombophilia).AcronymsSeeAPCR |