RELATIONSHIP BETWEEN VASCULAR ENDOTHELIAL CELL INJURIES AND RED CELL FRAGMENTATION SYNDROME

血管内皮细胞损伤与红细胞破碎综合征之间的关系

• 批准号: 06671086
• 负责人: WADA Hideo
• 金额: $ 1.41万
• 依托单位: MIE UNIVERSITY
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for General Scientific Research (C)
• 财政年份: 1994
• 资助国家: 日本
• 起止时间: 1994 至 1995
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-06671086/
• 关键词: RCFS; HUS; TTP; Vascular endothelial cell injuries; Vascular endothelial cell; Thrombomodulin

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.

红细胞分裂综合征（RCFS）包括血栓性血小板减少性紫癜（TTP）和药物性溶血性尿毒症综合征（HUS）具有很高的死亡率。血管内皮细胞标志物血栓调节蛋白（TM）在TTP和溶血性尿毒综合征患者中显著升高，在药物性溶血性尿毒综合征患者中略有升高。血浆细胞素水平显著升高，且与临床病程密切相关。RCFS患者呈高凝、低纤溶状态，大部分TTP患者血管性血友病因子高分子量多聚体降低，血小板活化。RCFS患者血浆组织因子途径抑制剂（TFPI）水平显著降低。在TTP患者血浆培养的人脐静脉内皮细胞中，MTT法吸光明显降低，BrDu法DNA合成增加，趋化性降低，组织因子（tissue factor， TF）活性升高，提示有多种因素激活或损伤血管内皮细胞。在用丝分裂霉素C或其衍生物培养的这些细胞中，MTT检测的吸光度、TF活性和趋化性降低。药物性溶血性尿毒中毒引起血管内皮细胞损伤的机制可能与TTP不同。各种激活或损伤可能影响RCFS的发生。

项目成果

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Hideo Wada 等人：“治疗获得性溶血性贫血的血浆交换/自行治疗”日常实践和血液。 4. 61-66 (1994)

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Wakita Y.,et al: "Trcatnert of throabetic thecorhocytoperic purpara with plasoin excharge" Jpn.J.Apheresis. 14. (1995)

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Wada H.,et al.：“健康和疾病中内皮细胞中的组织因子表达。”