Protein C Deficiency

• Anticoagulation

Patients with symptomatic thrombosis can be treated with direct oral anticoagulants (DOACs) or warfarin. Patients with symptomatic thrombosis can be treated with direct oral anticoagulants (DOACs) or warfarin.

When initiating warfarin therapy, it is important for clinicians to use therapeutic doses of heparin or low molecular weight heparin for initial anticoagulation and avoid When initiating warfarin therapy, it is important for clinicians to use therapeutic doses of heparin or low molecular weight heparin for initial anticoagulation and avoidwarfarin loading doses. Instead, once patients are therapeutically anticoagulated with a parenteral agent, warfarin should be started at the estimated maintenance dose (eg, 5 mg daily) and parenteral anticoagulation with heparin should continue for at least 5 days and until the INR is should be started at the estimated maintenance dose (eg, 5 mg daily) and parenteral anticoagulation with heparin should continue for at least 5 days and until the INR is≥ 2.

Since protein C is a vitamin K–dependent protein and has a short half-life compared to factors II and X, discontinuation of parenteral anticoagulants such as unfractionated or low molecular weight heparin before factor II and X levels have fallen sufficiently (to 20 to 40% of normal activity) can precipitate warfarin skin necrosis. This complication can be avoided by using DOACs, which appear to be as effective as Since protein C is a vitamin K–dependent protein and has a short half-life compared to factors II and X, discontinuation of parenteral anticoagulants such as unfractionated or low molecular weight heparin before factor II and X levels have fallen sufficiently (to 20 to 40% of normal activity) can precipitate warfarin skin necrosis. This complication can be avoided by using DOACs, which appear to be as effective aswarfarin for prevention of venous thromboembolism in patients with protein C deficiency. When using dabigatran or edoxaban, initial parenteral anticoagulation with unfractionated for prevention of venous thromboembolism in patients with protein C deficiency. When using dabigatran or edoxaban, initial parenteral anticoagulation with unfractionatedheparin or low molecular weight heparin for at least the first 5 days is recommended (1, 2). Since DOACs have a short onset of action, they can be started when heparin is stopped without a period of overlap of the 2 drugs.

Neonatal purpura fulminans is a form of deficiency that is fatal without replacement of protein C (using normal plasma or purified concentrate) along with anticoagulation with heparin or low molecular weight heparin.Neonatal purpura fulminans is a form of deficiency that is fatal without replacement of protein C (using normal plasma or purified concentrate) along with anticoagulation with heparin or low molecular weight heparin.