This website is intended for US healthcare professionals only

Epidemiology of SCPCD: incidence and prevalence of SCPCD

Epidemiology of SCPCD

Incidence and prevalence

Severe Congenital Protein C Deficiency (SCPCD) is a rare autosomal recessive disorder,1 and estimates of its incidence and prevalence vary.

Epide Desktop

The predicted prevalence of SCPCD is low, ranging from 1 in 40,000 to 250,000 people.3

Potential explanations for the low number of identified individuals include high fœtal mortality, death before diagnosis, under-diagnosis and under-reporting.1,3

Consanguinity, highly prevalent in the Middle East, Northern Africa and South and Western Asia,4 seems to play an important role in the incidence of SCPCD. Children born of consanguineous parents have a significantly higher risk of developing some genetic diseases compared with children born of non-consanguineous unions.5,6 Parental consanguinity is a common factor associated with people suffering from SCPCD.1


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

  2. Marlar RA, Mastovich S. Hereditary protein C deficiency: a review of the genetics, clinical presentation, diagnosis and treatment. Blood Coagul Fibrinolysis. 1990;1(3):319-330.

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

  4. Bener A, Mohammad RR. Global distribution of consanguinity and their impact on complex diseases: Genetic disorders from an endogamous population. Egyptian Journal of Medical Human Genetics. 2017;18(4):315-320.

  5. Shawky RM, et al. Consanguinity and its relevance to clinical genetics. Egyptian Journal of Medical Human Genetics. 2013;14(2):157–164.

  6. Tadmouri GO, et al. Consanguinity and reproductive health among Arabs. Reproductive Health. 2009;6(17):1–9.

SCPCD - Key Management info  

Interested in Severe Congenital Protein C
Deficiency (SCPCD) and its management?
Sign up below!

Sorry! There are some errors below that need to be fixed.
There seems to have been an error when sending the form.

You have the right to opt out of receiving such electronic communications and/or our newsletter, at any time, by using the opt out link in the communication or by contacting us at or by calling 1-866-888-0660. For more information on how Takeda processes your personal data, please refer to our Privacy Notice.

Please include @ in your email address


Thank you for submitting your details

You are about to leave
Continue Cancel