Activated protein C resistance | |
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Classification and external resources | |
ICD-9 | 289.81 |
OMIM | 188055 |
MeSH | D020016 |
Activated protein C resistance is a hemostatic disorder characterized by a poor anticoagulant response to activated protein C (APC). This results in an increased risk of venous thrombosis, which can cause heart attacks, strokes, and other problems with circulation.[1]
The disorder can be acquired or inherited, the hereditary form having an autosomal dominant inheritance pattern.[2]
Activated protein C (with protein S as a cofactor) degrades Factor Va and Factor VIIIa. Activated protein C resistance is the inability of protein C to cleave Factor Va and/or Factor VIIIa, which allows for longer duration of thrombin generation and may lead to a hypercoagulable state. This may be hereditary or acquired.[3] The best known and most common hereditary form is Factor V Leiden. Acquired forms occur in the presence of elevated Factor VIII concentrations.
Up to 64% of patients with venous thromboembolism might have activated protein C resistance.[4]
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