Activated protein C resistance

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ICD10 =
ICD9 = ICD9|289.81
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OMIM = 188055
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MeshID = 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. [cite journal |author=Dahlbäck B |title=The discovery of activated protein C resistance |journal=J. Thromb. Haemost. |volume=1 |issue=1 |pages=3–9 |year=2003 |pmid=12871530 |doi=]

The disorder can be acquired or inherited, the hereditary form having an autosomal dominant inheritance pattern. [cite journal |pmid=7902898 |year=1993 |month=Dec |author=Koster T, Rosendaal FR, De Ronde H, Briët E, Vandenbroucke JP, Bertina RM |title=Venous thrombosis due to poor anticoagulant response to activated protein C: Leiden Thrombophilia Study |volume=342 |issue=8886-8887 |pages=1503–6 |issn=0140-6736 |journal=Lancet]

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. [cite journal |author=Nicolaes GA, Dahlbäck B |title=Congenital and acquired activated protein C resistance |journal=Semin Vasc Med |volume=3 |issue=1 |pages=33–46 |year=2003 |pmid=15199491 |doi=10.1055/s-2003-38331] 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

Up to 64% of patients with venous thromboembolism might have activated protein C resistance. [cite journal |author=Sheppard DR |title=Activated protein C resistance: the most common risk factor for venous thromboembolism |journal=J Am Board Fam Pract |volume=13 |issue=2 |pages=111–5 |year=2000 |pmid=10764192 |doi=]

References