Thromb Haemost 1988; 59(01): 018-022
DOI: 10.1055/s-0038-1642558
Review Article
Schattauer GmbH Stuttgart

The Frequency of Type I Heterozygous Protein S and Protein C Deficiency in 141 Unrelated Young Patients with Venous Thrombosis

C L Gladson
The Department of Immunology, Research Institute of Scripps Clinic, La Jolla, California, USA
I Scharrer
The Department of Immunology, Research Institute of Scripps Clinic, La Jolla, California, USA
*   Zentrum der Inneren Medizin, Klinikum der Johann-Wolfgang-Goethe-Universität, Frankfurt, West-Germany
V Hach
The Department of Immunology, Research Institute of Scripps Clinic, La Jolla, California, USA
*   Zentrum der Inneren Medizin, Klinikum der Johann-Wolfgang-Goethe-Universität, Frankfurt, West-Germany
K H Beck
The Department of Immunology, Research Institute of Scripps Clinic, La Jolla, California, USA
*   Zentrum der Inneren Medizin, Klinikum der Johann-Wolfgang-Goethe-Universität, Frankfurt, West-Germany
J H Griffin
The Department of Immunology, Research Institute of Scripps Clinic, La Jolla, California, USA
› Author Affiliations
Further Information

Publication History

Received 09 October 1986

Accepted after revision 21 September 1987

Publication Date:
18 April 2018 (online)


The frequency of heterozygous protein C and protein S deficiency, detected by measuring total plasma antigen, in a group (n = 141) of young unrelated patients (<45 years old) with venous thrombotic disease was studied and compared to that of antithrombin III, fibrinogen, and plasminogen deficiencies. Among 91 patients not receiving oral anticoagulants, six had low protein S antigen levels and one had a low protein C antigen level. Among 50 patients receiving oral anticoagulant therapy, abnormally low ratios of protein S or C to other vitamin K-dependent factors were presented by one patient for protein S and five for protein C. Thus, heterozygous Type I protein S deficiency appeared in seven of 141 patients (5%) and heterozygous Type I protein C deficiency in six of 141 patients (4%). Eleven of thirteen deficient patients had recurrent venous thrombosis. In this group of 141 patients, 1% had an identifiable fibrinogen abnormality, 2% a plasminogen abnormality, and 3% an antithrombin III deficiency. Thus, among the known plasma protein deficiencies associated with venous thrombosis, protein S and protein C. deficiencies (9%) emerge as the leading identifiable associated abnormalities.

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