Table 4.

Therapeutic options for severe (homozygous or mixed heterozygous) protein C deficiency

• Plasma-derived protein C zymogen concentrates

(Ceprotin®, Protexel®)

60–80 IU/kg body weight slow i.v. bolus every 6 hours

(maximum rate 2 mL/min in adults or 0.2 ml/min in children <10 kg)

Target: post-infusion plasma protein C activity 1.0 IU/mL, pre-infusion plasma protein C activity >0.25 IU/mL

Alternative regimen:

100 U/kg slow i.v. bolus, followed by 10 IU/kg/h continuous infusion

Target: plasma protein C activity 1.0 IU/mL

• Fresh frozen plasma

20–30 mL/kg i.v. every 6 hours

• Activated human protein C – drotrecogin alpha activated (Xigris®)

20 μg/kg/hour for >10 hours?

not approved for substitution therapy of congenital protein C deficiency

After symptoms have resolved:

Vitamin K antagonist therapy – warfarin

Slow initiation, overlapping with protein C substitution