|Budget Amount *help
¥2,200,000 (Direct Cost : ¥2,200,000)
Fiscal Year 1995 : ¥800,000 (Direct Cost : ¥800,000)
Fiscal Year 1994 : ¥1,400,000 (Direct Cost : ¥1,400,000)
Red cell fragmentation syndrome (RCFS) including thrombotic thrombocytopenic purpura (TTP) and drug induced hemolytic uremic syndrome (HUS) have a high mortality. Vascular endothelial cell markers as thrombomodulin (TM) were significantly increased in patients with TTP and those with HUS,and these markers were slightly increased in patients with drug induced HUS.Plasma cytokyne levels were significantly increased and these were well correlated with clinical course. The patients with RCFS were hypercoagulableand hypofibrinolytic state, and decreased high molecular weight multimer of von Willebrand factor and activation of platelets were observed in most of TTP patients. Plasma level of tissue factor pathway inhibitor (TFPI) were significantly decreased in patients with RCFS.In human umbilical venous endothelial cell cultured with plasma of TTP patients, the absorbance of MTT assay was significantly decreased, and DNA synthesis was increased by BrDu assay, and chemotaxis was decreased, and tissue factor (TF) activity was increased, suggesting that there were several factors activating or injuring vascular endothelial cells. In thses cells cultured with mitomicin C or its derivative, the absorbance of MTT assay, TF activity, and chemotaxis reduced. The mechanism of vascular endothelial cell injuries in drug induced HUS might be different from those in TTP.Various activation or injuries might effect the onset of RCFS.