SummaryThe abnormal fibrinogen Haifa is characterized by the fact that calcium present during enzymatic digestion by plasmin does not protect the Haifa D gamma chain against further plasmin attack as it does in normal molecules.Since calcium binding to fibrinogen, ADP - platelet aggregation cofactor activity and gamma dimerization process induced by factor XIIIa are normal for fibrinogen Haifa, the corresponding sequences in the gamma chain are not involved. It seems rather that the anomaly resides near the gamma 302 plasmin cleavage site that is protected when calcium is bound to the gamma chain and that this affects the availability of the polymerization site located in the C terminal part of the chain.

Venous thrombosis, including superior and inferiorvena caval occlusion is considered an integral manifestation of Behçet’s disease. In this series, 5 of 11 patients (45.4%) with active Behçet’s disease had clinical evidence of present or past venous thrombosis. The mean euglobulin lysis time in the 11 subjects was found to be within the normal range: however, in 3 patients, significant impairment of fibrinolytic activity was demonstrated. Inhibition of systemic fibrinolysis may be a contributory factor in the pathogenesis of the thrombotic diathesis characteristic of Behçet’s disease.

Disseminated Intravascular coagulation may be an important and determining factor in the pathophysiology of some of the cutaneous vascular manifestations. The use of heparin in this condition seems to be the therapy of choice and sometimes it is life saving. Hence, the importance of being aware of the possibility and polymorphism of this phenomenon. Three cases are described where the presenting symptoms of the disease were mainly dermatological.

No abstract