Management of Severe Congenital Protein C Deficiency with Continuous Subcutaneous Infusion via Insulin Pump: A...

Severe congenital Protein C deficiency (SCPCD) is a rare autosomal recessive thrombophilia that typically presents in the neonatal period with early-onset life-threatening thrombotic complications. We report the case of a female infant who presented at birth with digital ischemic necrosis and laboratory evidence of consumptive coagulopathy consistent with neonatal purpura fulminans. Severe Protein C deficiency was confirmed by markedly reduced Protein C activity (<0.03 IU/mL) and compound heterozygous variants in the PROC gene. After initial stabilization and intermittent intravenous Protein C replacement, pharmacokinetic assessment showed marked peak-trough variability. Continuous subcutaneous infusion of Protein C concentrate was therefore initiated using a programmable insulin pump in combination with oral anticoagulation. This strategy achieved stable Protein C activity levels, allowed progressive reduction of the weight-adjusted replacement dose, and enabled removal of the central venous catheter. Continuous subcutaneous infusion of Protein C concentrate via an insulin pump, combined with oral anticoagulation, may represent a feasible long-term therapeutic option in selected patients with SCPCD.