血栓性血小板减少性紫癜和溶血性尿毒症综合征肾脏病理特点分析

Clinico-pathologic analysis of seven cases with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome

目的探讨血栓性血小板减少性紫癜和溶血性尿毒症(TTP/HUS)综合征的临床与肾脏病理特点。方法回顾性分析7例TTP/HUS综合征患者的临床特点并总结其中3例的肾脏病理特征。结果临床表现包括血小板减少6例、微血管病性溶血性贫血7例、发热6例、神经系统损害6例和急性肾功能衰竭7例。肾损害表现包括无尿3例,肉眼血尿4例,镜下血尿3例,蛋白尿7例,为(2.5±1.9)g/L。肾脏组织学检查3例,主要表现为肾内动脉血栓栓塞、肾小动脉内膜增厚、肾小球毛细血管微血栓形成、肾小球弥漫性内皮细胞增生和系膜细胞增生。死亡1例,发展为尿毒症3例,遗留慢性肾损害3例。发病年龄小、肾病理损害轻、给予血浆置换治疗患者,短期预后相对较好。结论TTP/HUS综合征是临床急症,预后较差。肾组织学检查有助于预后评价和指导治疗。

Objective To analyze the clinical and pathologic features of seven cases with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome（TTP/HUS syndrome）. Methods The clinical features of seven patients （five males and two females, aged 7-70 years） with TTP/HUS syndrome who were admitted to Peking Union Medical College Hospital from 1983 to 2004 and the pathologic features of three cases of them were analyzed. The possible causes included systemic lupus erythematosus, drug adverse reactions and upper respiratory infection. Results All patients presented the typical characteristics of microangiopathic hemolytic anemia and six suffered from thrombocytopenia. Six patients suffered from different degrees of fever and neurological impairment. All patients suffered from acute renal failure and four were anuria. Four had gross hematuria and the others had obvious microscopic hematuria. All had proteinuria, （2.5±1.9）g/L. Renal biopsy were taken in three of these patients. Thmmbo-embolism formation of the arteries and capillaries in the kidney, intimal thickening of the arterioles, diffuse proliferation of endothelial cells and mesangial cells were demonstrated. One patient died and three suffered from end stage renal disease and the others suffered from different degree of chronic renal lesions. The patients who were young or had slight renal pathologic lesion or were treated with plasma exchange, were likely to have relatively better short-term prognosis. Concolusions TTP/HUS syndrome is a severe syndrome with poor prognosis. Renal histological examination is useful to determine the treatment and to evaluate the prognosis.