HEREDITARY PROTEIN-S DEFICIENCY AND VENOUS THROMBOEMBOLISM - A STUDY IN 3 DUTCH FAMILIES

  • 1 January 1985
    • journal article
    • research article
    • Vol. 53  (2) , 273-277
Abstract
Protein S, a vitamin K-dependent coagulation factor, is involved in the regulation of the anticoagulant activity of activated protein C. Using an immunoradiometric assay for total protein S in plasma 14 patients (7 male and 7 female) in 3 unrelated Dutch families were identified as fulfilling the criteria for an isolated protein S deficiency. In 9 patients who were not receiving oral anticoagulant treatment the mean total protein S antigen concentration was 0.50 .+-. 0.08 U/ml (.+-. S.D.) and the calculated free protein S concentration was 0.15 .+-. 0.01 U/ml (.+-. S.D.). In the 5 patients who were on oral anticoagulant treatment the mean total protein S antigen was 0.23 .+-. 0.05 U/ml (.+-. S.D.). Seven of the 14 patients had a history of venous thromboembolism occurring at a mean age of 25 yr and often without an apparent cause. Protein S deficiency is inherited as an autosomal dominant trait.