SCPCD action tool
SUGGESTED ACTIONS WHEN FACING PURPURA FULMINANS
This is an action tool that has been created by Shire and is based on varying clinical practices and publications
For information only. Treatment decisions should always be based on the label of approved medications, and clinical training and expertise.
*There are other coagulation disorders that contribute to similar symptoms; #Steps 1 and 2 should be performed in parallel;
**Please refer to “Options for management of SCPCD” page of this website for treatment options; # Rarely, patients with moderate protein C activity have also presented with neonatal PF; ##Generic analysis needs to be done to confirm SCPCD.
***Genetic analysis needs to be done to confirm SCPCD.
FFP=Fresh frozen plasma; HB=Haemoglobin; INR=International Normalized Ratio; OAC=Oral anticoagulation; PC=Protein C; PF=Purpura fulminans; PS=Protein S; SCPCD=Severe congenital protein C deficiency; WCC= White cell count.
Napolitano LM, et al. Sepsis 2018: Definitions and Guideline Changes. Surg Infect. 2018;19:117-125.
Knoebl PN. Blood coagulation and inflammation in critical illness: the importance of the protein C pathway. UNI-MED Verlag; 2008.
Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Arch Dis Child. 2011;96:1066-1071.
Goldenberg NA, et al. Protein C deficiency. Haemophilia. 2008;14:1214-1221.
Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-322.
Khor B, et al. Laboratory tests for protein C deficiency. Am J Hematol. 2010;85:440-442.
Libourel EJ, et al. Protein C/S ratio, an accurate and simple tool to identify carriers of a protein C gene mutation. Br J Haematol. 2002;118:615-618.
Tcheng WY, et al. Severe congenital protein c deficiency: description of a new mutation and prophylactic protein C therapy and in vivo pharmacokinetics. Pediatr Hematol Oneal. 2008; 30: 166-71.