ICD-9-CM 289.81 MeSH D020016 | OMIM 188055 | |
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Activated protein C resistance (APCR) 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 problems with circulation, such as pulmonary embolism.
Contents
The disorder can be acquired or inherited, the hereditary form having an autosomal dominant inheritance pattern.
Pathophysiology
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. The best known and most common hereditary form is Factor V Leiden. Acquired forms occur in the presence of elevated Factor VIII concentrations.
Associated conditions
An estimated 64 percent of patients with venous thromboembolism may have activated protein C resistance.