Severe hypofibrinogenemia associated with bilateral ischemic necrosis of toes and fingers.

Severe hypofibrinogenemia was found in an Algerian woman who, since the age of 37 years, suffered three different episodes of ischemic necrosis of the toes and fingers leading to amputation of the toes and surgical removal of necrotic tissue (necretomy). No anti-fibrinogen antibody was present. The deficiency appeared to be due to severe congenital hypofibrinogenemia since the fibrinogen level remained at the same low level over a long period, without any abnormality of other coagulation proteins. The thrombotic events may be explained by the increased thrombin generation observed in the patient's plasma, due to the lack of thrombin adsorption onto a fibrin net.

Behçet syndrome associated with protein S deficiency.

Behçet syndrome is a multisystem disorder characterized by ocular, mucocutaneous, articular, gastrointestinal and neurologic abnormalities. We report here an unusual case of Behçet syndrome, characterized by the importance of the thrombotic events (7 phlebitis of both legs and resection of two toes). Additional manifestations of the Behçet syndrome occurred only 10 years after the first thrombotic episode. The oldest daughter of the propositus and his brother suffered also from thrombophlebitis; this familial history of thrombosis led to the performance of a haemostatic study. A congenital protein S deficiency was found in the propositus and in three of his children. Normal protein S levels were found in nine unrelated patients with Behçet syndrome. Thus this observation suggests that, when thrombotic manifestations are the first and major symptom of Behçet syndrome, an additional cause of thrombosis has to be investigated.