ACQUIRED SEVERE PROTEIN S-DEFICIENCY COMB INED WITH VENOUS THROMBOSISAFTER FEBRILE INFECTION IN A 7-YEAR-OLD GIRL

The importance of the anticoagulant properties of protein S is illustr ated by the high incidence of thromboembolic events in individuals wit h protein S-deficiency. A 7 year old girl was hospitalized with purpur a-like bruises and lesions on both tighs after she had suffered from f ebrile infection. A subsequently developing thrombosis of the left V. femoralis was treated successfully with urokinase. Haemostaseological investigations showed no signs of disseminated intravascular coagulati on. However, isolated severe degradation of all protein S-components d ue to the presence of a circulating autoantibody to protein S was foun d. After several months the antibody was detectable not any more, acti vity and antigens of protein S were normal.