Treatment of Homozygous Protein C Deficiency and Neonatal Purpura Fulminans with a Purified Protein C Concentrate

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Abstract
PROTEIN C is a vitamin K—dependent glycoprotein circulating in plasma as an inactive zymogen.1 It is converted to the serine protease—activated protein C, a natural anticoagulant that inactivates factors V and VIII after they are activated by thrombin during the coagulation process.2 , 3 The homozygous and double heterozygous forms of congenital protein C deficiency can result in life-threatening neonatal thrombosis and purpura fulminans, diseases that are frequently associated with disseminated intravascular coagulation.4 , 5 At least 19 cases have been reported.4 , 6 7 8 9 10 11 The predominant clinical signs of purpura fulminans consist of ecchymotic skin lesions that spontaneously become necrotic.12 , 13 We describe the case of an infant with severe protein C deficiency and neonatal purpura fulminans who was treated successfully with a highly purified concentrate of protein C. Long-term therapy with the concentrate was well tolerated.