Protein C substitution in sepsis-associated purpura fulminans

Objective: To assess the effect of protein C (PC) substitution on imminent peripheral necroses and overall outcome in patients with sepsis-associated purpura fulminans. Design: Case series, Setting: Intensive care units of two university hospit als. Patients: A total of 12 patients with purpura fulminans, disseminated intra vascular coagulation and imminent peripheral necroses in association with s epsis caused by Neisseria meningitidis (n = 5), Streptoccccus pneumoniae (n = 2), Capnocytophaga canimorsus (n = 2), and Staphylococcus aureus (n = 1) , In two patients, no pathogens were identified. Interventions: Intravenous administration of PC concentrate (100 lU/kg ever y 6 hrs), In addition, antithrombin III substitution, antimicrobial therapy , hemodynamic support, and mechanical ventilation in all patients and hemod iafiltration in 10 patients. Main Results:After the onset of PC, progressive peripheral ischemia was rev ersed irrespective of the etiology of infection, Laboratory variables refle cting disseminated intravascular coagulation improved rapidly, although the recovery of the platelet count was retarded in the patients who subsequent ly died. No drug-related adverse events were noted. Amputations were necess ary in two patients, and necrotic tips of fingers and toes were macerated i n a third. The hospital mortality was 42%. Of the five lethal cases, two we re caused by S. pneumoniae, one by ill. meningitidis, one by C. canimorsus, and one by an unknown pathogen. Conclusions: This article provides encouraging results on the use of PC sub stitution in meningococcal purpura and presents new data on the administrat ion of this drug to patients with septic purpura caused by other bacterial species, By clinical judgment, PC limited the extent of tissue necrosis. Th e small number of patients does not allow for any conclusions on the potent ial effect of PC on mortality. A controlled and randomized study with a lar ger number of patients is needed before any recommendations can be given on the use of PC in sepsis-related purpura fulminans and shock.