Supriya Ghosh

Activated protein C resistance

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ICD-9-CM  289.81
MeSH  D020016
OMIM  188055
Activated protein C resistance

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.

References

Activated protein C resistance Wikipedia


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