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protein C

B01AD12

Forms & Strengths

Lyophilized powder for reconstitution: 500 IU, 1000 IU per vial

Adult Dosing

Congenital thrombotic purpura: Initial 40-80 IU/kg IV bolus, followed by maintenance 45-60 IU/kg every 6 hours
Loading dose calculated to raise plasma protein C activity to normal levels (100%)

Pediatric Dosing

Congenital thrombotic purpura / fulminant purpura in neonates and children: 40-80 IU/kg IV initial, tailored to maintain trough levels >25-50%

Indications

Treatment and prophylaxis of venous thrombosis and purpura fulminant in patients with severe congenital protein C deficiency
Short-term prophylaxis when initiating coumarin therapy in congenital deficiency

Mechanism of Action

Replaces deficient natural anticoagulant protein C
Activated protein C (aPC) proteolytically inactivates coagulation factors Va and VIIIa in the presence of calcium and phospholipid, downregulating thrombin generation

Contraindications

Known life-threatening hypersensitivity to protein C concentrate or bovine/murine proteins (if derived from plasma/expression systems containing them)
Concurrent warfarin-induced skin necrosis risk if not overlapped properly (though used to treat/prevent it)

Adverse Reactions

Hypersensitivity / allergic reactions (rash, urticaria, tightness in chest)
Hemorrhage / bleeding complications
Fever, dizziness, nausea
Development of neutralizing antibodies (inhibitors)

Drug Interactions

Anticoagulants (heparin, oral anticoagulants): Increased risk of bleeding when used concomitantly
Fibrinolytic agents: Potential increased risk of hemorrhage

Curated Content: Needs Vetting Before Put to Clinical Use