Kaitlin Nutley, BCCCP, PharmD
Critical Care Pharmacist
UAB Hospital - University of Alabama At Birmingham
Birmingham, Alabama
Disclosure information not submitted.
Christopher Leong, PharmD, BCCCP
Critical Care Pharmacist
Northwestern Memorial Hospital, United States
Disclosure information not submitted.
Joseph Bailey, MD
Pulmonary and Critical Care Medicine Fellow
Northwestern Memorial Hospital, United States
Disclosure information not submitted.
Title: Protein C concentrate in sepsis acquired purpura fulminans
Case Report Body:
Introduction: Purpura fulminans is a life threatening and highly disabling condition characterized by rapidly evolving thrombosis in the dermal and systemic microcirculation. Sepsis acquired purpura fulminans (SAPF) is most commonly caused by N. meningitidis, Group A and B streptococci, and S. pneumoniae infections. There are few therapeutic options that have been found to reduce morbidity for SAPF, and consensus is lacking regarding treatment options. The goal of treatment involves pathogenesis targeting approaches that generally aim at the restoration of balance of the pro- and anti-coagulation pathways. Protein C concentrate has been used in congenital purpura fulminans, but is less well described in adults diagnosed with SAPF.
Description: A 51 year-old male with no significant past medical history was admitted for septic shock secondary to Streptococcus pyogenes bacteremia. On presentation he had abnormal liver function tests and coagulation labs. On day two of admission, he developed purpuric lesions on his hands and legs, which rapidly progressed to severe ischemic injury to the extremities. Hematology was consulted to provide recommendations for transfusion requirements and they recommended initiation of protein C concentrate. A baseline protein C level was ordered and pharmacy was consulted for assistance of dosing and feasibility of obtaining the drug. Protein C concentrate was initiated with the following regimen: 100 units/kg bolus followed by a maintenance regimen of 45 units/kg every six hours and was continued for a total of 5 days. Over the next few days, the patient’s platelet count, coagulation labs, and protein C levels improved. His baseline protein C level one day prior to therapy was low at 25% and improved to 320% on the final day of therapy. The patient required multiple amputations of the affected extremities, but recovered with excellent cognitive status.
Discussion: Currently there are no evidence-based treatment guidelines or direct comparative evaluation of the efficacy of the different therapeutic approaches in the treatment of SAPF. Protein C concentrate is a possible treatment option; however, optimal dosing and treatment duration remains unknown. We provide additional information regarding dosing, duration, lab monitoring and feasibility on the use of protein C concentrate in SAPF.