[Plasma level of various markers in patients with thrombotic thrombocytopenic purpura]

We examined various markers in 17 patients with thrombotic thrombocytopenic purpura (TTP), 13 with red cell fragmentation syndrome (RCFS) induced by mitomycin C, and 36 with RCFS caused by disseminated intravascular coagulation (DIC). A platelet activating factor and decrease of von Willebrand factor were frequently observed in patients with TTP, but were not in those with RCFS due to either mitomycin C or DIC. Since increased vascular endothelial cell markers and decreased MTT assay were observed in patients with TTP, it is considered that vascular endothelial cell injury is associated with patients with TTP. The increased plasma cytokines and the hypercoagulable and hypofibrinolytic state indicates that the vascular endothelial cell injury might be caused by microthrombus or activated immune system. Although patients with TTP were treated with plasma exchange, anti-platelet therapy, and high dose steroid therapy, the mortality was still high.