Severe Congenital Protein C Deficiency
Several laboratory tests are recommended upon signs of purpura fulminans:
- Given possible confounding factors, protein C deficiency should be confirmed using multiple methods2
PROTEIN C (PC) ACTIVITY ASSAY1,3
Protein S assay1 (low PC levels but normal Protein S levels are suggestive of SCPCD)
Protein C antigen5
Prothrombin time (PT)2,5
Activated partial prothrombin time2,5
Protein C level testing in parents1 (Protein C activity assay and Protein C antigen)
Warnings and Precautions
Hypersensivity: CEPROTIN may contain trace amounts of mouse protein and/or heparin as a result of the manufacturing process. Allergic reactions to mouse protein and/or heparin cannot be ruled out. If symptoms of hypersensitivity/allergic reaction occur, discontinue the injection/infusion. In case of anaphylactic shock, the current medical standards for treatment are to be observed.
Transmission of infectious agents: Because CEPROTIN is made from human plasma, it may carry a risk of transmitting infectious agents, e.g. viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and theoretically, the Creutzfeld-Jakob disease (CJD) agent.
Bleeding episodes: Several bleeding episodes have been observed in clinical studies. Concurrent anticoagulant medication may have been responsible for these bleeding episodes. However, it cannot be completely ruled out that the administration of CEPROTIN further contributed to these bleeding events. Simultaneous administration of CEPROTIN and tissue plasminogen activator (tPA) may further increase the risk of bleeding from tPA.
Heparin-induced thrombocytopenia (HIT): CEPROTIN contains trace amounts of heparin which may lead to HIT, which can be associated with a rapid decrease of the number of thombocytes. If HIT is suspected, determine the platelet count immediately and consider discontinuation of CEPROTIN.
Low sodium diet/Renal impairment: Patients on a low sodium diet or who have renal impairment should be informed that the quantity of sodium in the maximum daily dose of CEPROTIN exceeds 200 mg. Monitor patients with renal impairment closely for sodium overload.
Common adverse reactions related to CEPROTIN observed in clinical trials were hypersensitivity or allergic reactions: lightheadedness, itching and rash.
CEPROTIN [Protein C Concentrate (Human)] is indicated for neonates, pediatric and adult patients with severe congenital Protein C deficiency for the prevention and treatment of venous thrombosis and purpura fulminans.
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Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071
Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.
Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.
Libourel, EJ, et al. Protein C/S ratio, an accurate and simple tool to identify carriers of a protein C gene mutation. British Journal of Haematology. 2002;118:615-618.
Khor, B et al. Laboratory tests for protein C deficiency. Am. J. Hematol. 2010;85:440-442.
Tairaku S, et al. Prenatal genetic testing for familial severe congenital protein C deficiency. Hum Genome Var. 2015;2:15017.