Protein C Concentrate Added to Standard Therapy May Reduce Mortality Risk in Sepsis-Induced Purpura Fulminans

Despite an extremely poor prognosis, eight consecutive patients with acute-onset purpura fulminans (PF) secondary to systemic bacterial infection survived following treatment with a licensed human plasma-derived protein C concentrate (Ceprotin, Baxter Healthcare) in conjunction with standard sepsis therapy. In a ninth patient with PF caused by heat shock, protein C infusion was halted after two days; he died after 10 days from refractory multi-organ failure. Coagulopathy and severe vasopressor-dependent sepsis were present in all nine patients, who were managed at three Austrian hospitals. Prior to initiation of protein C therapy, the predicted mortality was 100% and 78.3% (range 31% to 88.5%) in four children and five adults, respectively.

Protein C was given as an initial bolus infusion (100 U/kg) followed by continuous infusion at 10 U/kg/hour, adjusted to obtain a plasma protein C activity level of 1.0 U/mL. Platelet, red blood cell, fibrinogen and antithrombin products were administered as needed. Coagulopathy resolved within a few days in all eight patients, and organ function was completely restored without residual dysfunction. At a median follow-up of eight months, all patients were fully active without apparent limitations.

Currently, Ceprotin is approved only for congenital protein C deficiency. Given these very encouraging results and the known association between PF and breakdown of the protein C system, the investigators concluded that “controlled clinical studies are urgently needed to gain more scientific evidence for this potentially lifesaving, but still off-label therapy in patients with PF.”