Unexpected pulseless disease associated with recurrent venous thromboembolisms.

Venous thromboembolic disease is a well-documented complication of Klinefelter's syndrome, even if mechanisms underlying this prothrombotic state have not been conclusively established. On the contrary, arterial thrombosis is far less frequent, and a case of a patient with Klinefelter's syndrome presenting with simultaneous venous thrombtoembolic disease and a complete thrombosis of the left subclavian artery is presented. Elevated levels of type 1 plasminogen activator inhibitor, in the absence of other usual thrombophilic abnormalities, raise the question of the role played by this inhibitor of the fibrinolysis in the arterial and venous thromboses presented by this patient.