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Plasma protein S deficiency in familial thrombotic disease
HP Schwarz, M Fischer, P Hopmeier, MA Batard and JH Griffin
A family with a history of severe recurrent venous thromboembolic disease
was studied to determine if a plasma protein deficiency could account for
observed disease. Protein S levels in plasma were determined
immunologically using the Laurell rocket technique. The propositus, his
mother, his aunt, and his cousin who were clinically affected had 17% to
65% of the control levels of protein S antigen (normal range, 71% to 147%).
Since three of these patients were receiving oral anticoagulant therapy,
the ratios of protein S to prothrombin, factor X, and protein C in these
patients were compared with values for a group of orally anticoagulated
controls. These results suggested that protein S is half-normal in all
family members with thrombotic disease. Other proteins known to be
associated with familial thrombotic disease, including antithrombin III,
plasminogen, fibrinogen, and protein C, were normal. Because plasma protein
S serves as a cofactor for the anticoagulant activity of activated protein
C and because protein C deficiency is associated with recurrent thrombotic
disease, it is suggested that recurrent thrombotic disease in this family
is the result of an inherited deficiency of protein S.
Volume 64,
Issue 6,
pp. 1297-1300,
12/01/1984
Copyright © 1984 by The American Society of Hematology
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