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. ( J PEDIATR 1995; 126:646-52)