Summary
A cohort study was undertaken to compare the incidence of thrombosis in patients with
inherited deficiency of Antithrombin III (n = 9), Protein C (n = 36) and Protein S
(n = 36). The patients were stratified for schedule of antithrombotic prophylaxis
and followed for a total period of 160 patient-years. Seven venous thrombosis were
observed for a total incidence of 4.3% pts.-ys. The incidence of thrombosis was not
significantly different in patients of different age, sex and schedule of prophylaxis,
although there was a trend to a lower incidence in young individuals and in those
receiving long-term oral anticoagulation. Patients with AT III deficiency had an higher
incidence of thrombosis than patients with Protein C or Protein S deficiency (12 vs.
2.8 vs. 3.3% pts.-ys., p <0.05), despite the fact that they were, on average, younger
and more prophylaxed. This study suggests that congenital Antithrombin III deficiency
constitutes a greater risk of thrombosis than congenital deficiences of Protein C
and Protein S.