Replacement treatment with protein C in an 18-year-old man with meningococcal sepsis and purpura fulminans.

In separate studies on Neisseria meningitidis sepsis, Powars and Fiynvandraat suggested that low protein C levels may be responsible for disseminated intravascular coagulopathy and purpura fulminans. Following on this observation, we used protein C concentrate in an 18-year-old male patient with septic shock and purpura fulminans. The patient's coagulation parameters were seriously altered: AT 45%; protein C 21%; PT 50%; platelets 55000; D-dimer 2400. Early treatment included immediate administration of 3000 IU of antithrombin and intensive therapy: antibiotic therapy, volemic replacement, supported by inotropic drugs and oxygen therapy. Given the patient's low protein C levels and the progression of purpura, replacement therapy with protein C concentrate was instituted. The initial dose of 80 IU/kg/bw (5600 IU) in bolus, was adjusted according to blood laboratory values and then set at 2000 IU every 8 hours for 4 days. An increase in protein C was observed (78%) after the 1st administration, while the D-dimer levels fell by 50%. By day 7, the patient's cardiocirculatory conditions had stabilized and the coagulation parameters had normalized; the patient was discharged from the intensive care unit. Protein C replacement therapy normalized the coagulation parameters and blocked the evolution of the skin injuries.