Protein S Deficiency
There are three types of Hereditary Protein S Deficiency:
- Type I (quantitative deficiency) - decreased Protein S activity; decreased total Protein S; decreased free Protein S
- Type II (functional deficiency) - decreased Protein S activity; normal total Protein S; normal free Protein S
- Type III (normal total protein S levels) - decreased Protein S activity; normal total Protein S; decreased free Protein S
It is also possible to have an acquired deficiency of Protein S, which is seen in some cases of DIC, DVT, PE, pregnancy and liver disease and during treatment with anticoagulants such as warfarin.
Estimates
Protein S Deficiency occurs in about 1 in 20,000 people (1). Of those less than half develop thrombosis, usually between the ages of 20 and 30. Of all the inherited thrombophilic conditions, it remains the most difficult to diagnose (2).
Number of People in the UK with Protein S Deficiency:
- 1 in 20,000 of UK population (60m) -> 3000
Number of UK Cases of Protein S Deficiency With Thrombophilia:
- 3000 with PSD -> 2500 over 20 yrs old -> 40% affected -> 1000
References
- NIH Genetics Home Reference: ghr.nlm.nih.gov
- Medline Plus: medlineplus.gov
- Blood, Vol. 95, No. 6, 2000, Makris et al: www.bloodjournal.org
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Last updated: Saturday, 7th December 2019
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