Treatment of purpura fulminans in meningococcemia with protein C concentrate.

OBJECTIVE

To evaluate the clinical and laboratory effects of protein C concentrate as an adjunct to conventional therapy in the treatment of meningococcemia with purpura fulminans.

DESIGN

Case series (pilot study).

SETTING

Intensive care unit in a tertiary care pediatric hospital.

PATIENTS

Four children (aged 3 months to 15 years) requiring intensive treatment for meningococcemia with shock, disseminated intravascular coagulation, and purpura fulminans.

INTERVENTION

Intravenous administration of a protein C concentrate (100 IU/kg every 6 hours).

MAIN OUTCOME MEASURES

Plasma protein C amidolytic activity, fibrinogen, and D-dimers; evolution of skin and limb lesions.

RESULTS

Treatment with protein C concentrate led to a rise in plasma protein C activity levels to within normal limits in all patients, associated with an increase in plasma fibrinogen and a bimodal decrease in D-dimers. No adverse effects were noted. All patients had reversal of organ dysfunction despite the severity of the initial illness. Two patients recovered completely with no sequelae; two required amputations.

CONCLUSIONS

These encouraging clinical and laboratory results and the absence of side effects warrant the initiation of a double-blind, randomized controlled multicenter trial to determine the role of protein C replacement in the treatment of meningococcemia-associated purpura fulminans.