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Clinical signs and symptoms of Severe Congenital Protein C Deficiency (SCPCD)

SCPCD - Key Management Information

Key information for the management of
severe congenital protein C deficiency (SCPCD)


Etiology of SCPCD

SCPCD: a rare autosomal recessive disorder that can manifest as purpura fulminans, among other symptoms, as early 2-12 hours after birth.1,2

  • Caused by a mutation in the Protein C (PC) gene or PROC.SCPCD involves the following type of mutations:4

Learn more about
the causes of protein C deficiency

Learn more about
the genetics of SCPCD


Symptoms of SCPCD

Infants with SCPCD usually present within hours of birth with symptoms including:1,4,*

  • Purpura fulminans
  • Disseminated intravascular coagulation
  • Retinal vessel thrombosis
  • Venous thrombosis
  • Cerebral venous thrombosis

*The symptoms presented here are not an exhaustive list. Other symptoms may be present in the event of SCPCD.1,4
Learn more about
the signs and symptoms of SCPCD

SCPCD Diagnostic Tests
  • PC activity assay1,4
  • Protein S (PS) assay4
  • PC antigen5
  • PC/PS ratio shown here may a indicate a genetic cause6
  • PC level testing in parents (partial reduction of PC levels in parents is highly suggestive of SCPCD)4


A full array of tests are necessary to rule out acquired PC deficiency4

Learn more about
how to test for SCPCD
Learn more about
the differences between acquired
and congenital PC deficiency

Treatment options for SCPCD
  • PC replacement2,7
  • Anticoagulants2,7
  • Liver transplantation2,7


The chosen treatment depends on a variety of factors detailed in the available guidelines

Learn more about
managing SCPCD


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

  2. Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-322.

  3. Patracchini P, et al. Sublocalization of the human protein C gene on chromosome 2q13-q14. Hum Genet. 1989;81(2):191-192.

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

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

  6. 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.

  7. Kroiss S, Albisetti M. Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency. Biologics: Targets & Therapy. 2010;4:51–60.

SCPCD - Key Management info  

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