Protein S deficiency

Protein S deficiency is a hyperocoagulable state associated with increased risk of venous thrombosis (up to 5% of patients with deep venous thrombosis may carry this deficiency).

Epidemiology

Protein S deficiency may be expected in ≈1 of every 500 people 3.

Pathology

Protein S, a vitamin K-dependent physiological anticoagulant, acts as a non-enzymatic cofactor to activate protein C in the degradation of factor Va and factor VIIIa. Decreased (antigen) levels or impaired function of protein S leads to decreased degradation of factor Va and factor VIIIa and therefore an increased propensity to venous thrombosis.

Genetics

Most cases are caused by mutations in the PROS1 gene.

 

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Article information

rID: 57111
System: Haematology
Synonyms or Alternate Spellings:
  • Deficiency of protein S

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