Usefulness of PPSB^®-HT for severe congenital protein C deficiency

Abstract

Purpura fulminans (PF) associated with severe protein C deficiency rapidly progresses and often becomes severe. The human prothrombin complex concentrate (PPSB^®-HT), which contains appreciable amounts of protein C, can be used for home infusion therapy in hemophilia patients. We treated two patients with human-plasma-derived activated protein C concentrate (APC), but this treatment required frequent hospital admission, which put a great strain on them and their respective families. Therefore, we changed APC to PPSB^®-HT for home infusion, which provided an early interventional measure to prevent serious conditions. Pediatric patients with congenital protein C deficiency await the approval of PPSB^®-HT for use in hemophilia treatment.