Treatment of purpura fulminans in meningococcemia with protein C concentrate

Abstract

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.