The clinical analysis of 12 patients with thrombotic microangiopathy

目的 探讨血栓性微血管病(thrombotic microangiopathy, TMA)的临床特点和治疗,以加深对本病的认识.方法 回顾分析12例TMA患者的临床表现、实验室检查、治疗及预后.结果 12例患者主要临床表现为微血管性溶血性贫血(12/12),血小板减少(12/12),肾脏受损(12/12),神经精神症状(6/12),发热(7/12).8例进行了血浆置换(8/12),1例死于合并症(1/8),7例好转出院(7/8);余4例血浆输注(4/12),1例死于本病(1/4),3例好转出院(3/4).结论 TMA临床表现多样,病因复杂,起病急,病情危重,预后差;提高认识,及早诊治可以改善预后,降低死亡率.Objective To explore clinical characteristics and treatment of the thrombotic microangiopathy(TMA), to improve the recognition of TMA.Methods The clinical manifestations, laboratory examinations, therapeutic methods and prognosis of 12 TMA patients were analyzed retrospectively.Results The clinical manifestations were characterized by microangiopathic hemolytic anemia(12/12), thrombocytopenia(12/12), renal lesion(12/12), fever(7/12), and neurological abnormalities or mental disorders(6/12).Eight patients were treated by plasma exchange therapy, 1 patient died from a complication(1/8);7 patients were discharged with improved condition(7/8).Four of the other patients were treated by plasma transfusion(4/12), 1 patient died from TMA(1/4);3 patients were discharged with improved condition(3/4).Conclusion TMA is a severe acute syndrome with various clinical manifestations, complicated pathogenesis and high mortality.It is important to increase the awareness and provide early diagnosis of TMA in order to improve prognosis and reduce mortality.中国科学引文数据库(CSCD)6539-5423