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Thromb Haemost 1985; 53(02): 273-277
DOI: 10.1055/s-0038-1661292
Original Article
Schattauer GmbH Stuttgart

Hereditary Protein S Deficiency and Venous Thrombo-Embolism

A Study in Three Dutch Families
A W Broekmans
The Hemostasis and Thrombosis Research Unit, Department of Hematology, Leiden University Hospital, Leiden, The Netherlands
,
R M Bertina
The Hemostasis and Thrombosis Research Unit, Department of Hematology, Leiden University Hospital, Leiden, The Netherlands
,
J Reinalda-Poot
The Hemostasis and Thrombosis Research Unit, Department of Hematology, Leiden University Hospital, Leiden, The Netherlands
,
L Engesser
The Hemostasis and Thrombosis Research Unit, Department of Hematology, Leiden University Hospital, Leiden, The Netherlands
,
H P Muller
1   The Department of Internal Medicine, Majella Hospital, Bussum, The Netherlands
,
J A Leeuw
2   The Division of Pediatric Oncology, University Hospital Groningen, Leiden, The Netherlands
,
J J Michiels
3   The Department of Hematology, University Hospital Rotterdam, Leiden, The Netherlands
,
E J P Brommer
4   The Gaubius Institute, Health Research Division TNO, Leiden, The Netherlands
,
E Briët
The Hemostasis and Thrombosis Research Unit, Department of Hematology, Leiden University Hospital, Leiden, The Netherlands
› Author Affiliations
Further Information

Publication History

Received 18 October 1984

Accepted 06 February 1985

Publication Date:
18 July 2018 (online)

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Summary

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 we identified 14 patients (7 male and 7 female) in three unrelated Dutch families 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 five 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 years and often without an apparent cause. Protein S deficiency is inherited as an autosomal dominant trait.

Keywords

Hereditary protein S deficiency - Thrombotic disease - Oral anticoagulant treatment
 

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