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Testing for
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 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

Genetic analysis2

Protein C level testing in parents1 (Protein C activity assay and Protein C antigen)

  • PC testing in family members is recommended to determine if the deficiency is genetic and whether it is a homozygous or heterozygous mutation.2


Acute treatment of PF and VT with CEPROTINWhat is protein C deficiency?Discover more about SCPCD

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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.


  1. Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071

  2. Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.

  3. Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.

  4. 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.

  5. Khor, B et al. Laboratory tests for protein C deficiency. Am. J. Hematol. 2010;85:440-442.

  6. Tairaku S, et al. Prenatal genetic testing for familial severe congenital protein C deficiency. Hum Genome Var. 2015;2:15017.

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