[Subclinical pro-thrombotic state in a relapse of Behcet's disease--case report]

A patient, aged 34, with a relapse of Behcet's disease (BD) showed laboratory features of enhanced coagulation activation without any symptoms of arterial or venous thrombosis. Elevated levels of thrombin-antithrombin III complexes (TAT), prothrombin fragments 1 + 2 (F1 + 2), associated with impairement of fibrinolysis were found. Total fibrinolytic activity, measured on fibrin plates, plasma concentration of tissue-type plasminogen activator (tPA:Ag) and plasmin-alpha 2-antiplasmin complexes (PAP) were low, whereas plasminogen activator inhibitor 1 concentration (PAI-1:Ag) was elevated in comparison with normal values. There intravenous 500 mg doses of cyclophosphamide, given on day 0, 7 and 50, resulted in a marked clinical improvement. This was accompanied by the normalization of augmented thrombin generation and increased fibrinolytic activity. This is the first report to show prothrombotic tendency in a relapse of Behcet disease and beneficial effect of cyclophosphamide therapy on haemostatic abnormalities. A degree of activation of coagulative and fibrinolytic system seems to be of importance in the pathogenesis of Behcet's disease, and monitoring of hemostatic parameters could be helpful in clinical assessment.