ARTICLE
Association of Protein S Deficiency with Thrombosis in a Kindred with Increased Levels of Plasminogen Activator Inhibitor-1
Charles D. Bolan;
Chitra Krishnamurti;
Douglas B. Tang;
Leonthena R. Carrington; and
Barbara M. Alving
15 October 1993 | Volume 119 Issue 8 | Pages 779-785
Objective: A single kindred in North America with venous thrombosis was described as having defective fibrinolysis because of increased levels of plasminogen activator inhibitor-1 (PAI-1). Our study describes the discovery of protein S deficiency in this kindred and its association with venous thromboembolism.
Design: A family study.
Setting: Community.
Participants: Twenty-eight adults (ages 21 to 71 years) from three generations of the kindred; seven had a history of venous thromboembolism.
Measurements: Plasma levels of total and free protein S antigen, as well as the activities of protein S, protein C, PAI-1, and antithrombin III.
Results: Six of 7 persons (86%) with a history of venous thromboembolism were deficient in total and free protein S; of 21 asymptomatic members, 9 were deficient in protein S (P = 0.08). When compared with these 9 asymptomatic family members, the 6 persons with protein S deficiency and a history of thrombosis tended to smoke (P = 0.01) and to have higher triglyceride levels (P = 0.001). Overall, the mean PAI-1 activity in the 7 persons who had thrombosis was 7.9 kAU/L (AU/mL) and was 9.3 kAU/L (AU/mL) in the 21 persons who did not have thrombosis (95% CI, –9.9 to 7.0).
Conclusions: In this kindred, a deficiency of total and free or functional protein S is the cause of thrombosis. Measurement of PAI-1 activity was not useful in the evaluation of familial thrombosis. The utility of the routine measurement of PAI-1 activity in the evaluation of familial thrombosis has not been established.
Author and Article Information
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From the Walter Reed Army Institute of Research and the Walter Reed Army Medical Center, Washington, D.C.
Requests for Reprints: Charles D. Bolan, MD, Department of Hematology Oncology, Ward 78, Walter Reed Army Medical Center, Washington, DC 20307.
Acknowledgments: The authors thank Dr. Jose-Antonio Fernandez for measuring the plasma ß chain-C4BP concentration in the laboratory of Dr. John H. Griffin at the Scripps Research Institute in La Jolla, California.
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