activated protein C resistance


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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 function

activated 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).
References in periodicals archive ?
The limitations in the originally devised assay for activated protein C resistance led to the development of a modified assay.
Other variations of the activated protein C resistance assay have been developed.
For the first 6 months of life, the reference range for the original assay for activated protein C resistance was found to be higher than in individuals older than 6 months.
One method of reporting a result for activated protein C resistance involves the use of a normalized ratio.
The original assay, despite its limitations, is undergoing evaluation to determine whether the numerical test result for activated protein C resistance provides information regarding hypercoagulability in the absence of factor V Leiden by a DNA-based method.
Diagnostic Algorithm for Assessment of Activated Protein C Resistance and Factor V Leiden
Figure 2 shows an algorithm for the diagnosis of activated protein C resistance and the factor V Leiden mutation.
The assessment for activated protein C resistance is initiated by performance of a screening test using factor V-deficient plasma as a diluent for the patient plasma.
Factor V Arg 306 [right arrow] Gly mutation is not associated with activated protein C resistance and is rare in Taiwanese Chinese.
Lack of activated protein C resistance in healthy Hong Kong Chinese blood donors: correlation with absence of Arg506-Gln mutation of factor V gene [letter].
Activated protein C resistance assay performance: improvement by sample dilution with factor V--deficient plasma.
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