Table 5.

Therapeutic options for heterozygous protein C deficiency

Asymptomatic carrier

No long-term therapy

Prolonged / intensified prophylaxis of thromboembolic events (with LMWH) in risk situations

Patients with first thromboembolic event

Therapeutic (full-dose) anticoagulation with LMWH

Overlapping slow initiation of warfarin (target INR 2.0–3.0) for 6–12 months

Recurrent thromboembolic events

Therapeutic (full-dose) anticoagulation with LMWH

Overlapping slow initiation of long-term warfarin therapy (target INR 2.0–3.0)

Cases of coumarin-induced skin necrosis

Therapeutic (full-dose) anticoagulation with LMWH or UFH

Protein C concentrate (Ceprotin® initial dose 60 IU/kg every 6 h)

Target plasma protein C activity: post-infusion 1.0 IU/mL and pre-infusion

>0.25 IU/mL

Drotrecogin alpha activated (not approved)

overlapping slow initiation of warfarin therapy (target INR 2.0–3.0)

Abbreviations: INL, international normalized ratio; LMWH = low molecular weight heparin; UFH = unfractionated (standard) heparin.