摘要
目的分析不同病因的血栓性微血管病(TMA)患者的临床特点,血浆置换(PE)治疗效果及预后,以提高对这一组疾病及其治疗的认识。方法回顾性分析作者医院2005-2013年间收治的经过血浆置换治疗的血栓性微血管病患者的临床表现、实验室检查、治疗、随访及预后等资料。结果共22例患者,男:女比为9:13,平均年龄为33.1±13.8岁。2例为原发性TMA,病程呈良性,PE治疗效果好,长期存活率高;3例为系统性红斑狼疮相关TMA,肾脏损害重,需要肾脏替代治疗,PE治疗完全缓解率及存活率为33.3%。17例为造血干细胞移植术后TMA,肾脏损害相对轻,均不需要肾脏替代治疗,对PE总体反应率41.1%,完全缓解率为17.6%,但长期预后差,存活率仅为11.8%。结论血浆置换治疗对原发TMA治疗效果最好;继发于系统性红斑狼疮及造血干细胞移植的TMA治疗效果不佳。原发者PE可作为一线治疗;对常规治疗方法无效的继发性TMA,PE可作为二线治疗推荐。
Objective Analyze the clinical and laboratory characteristics,plasmapheresis response rate and outcomes in patients with thrombotic microangiopathy of different etiology.Methods Retrospectively review all the patients diagnosed as TMA and accepted therapeutic plasmapheresis during 2005 and 2013 at our center.Their clinical,laboratory characteristics,treatments,follow-ups and outcomes were investigated.Resuits Altogether there are 22 patients with 9 males and 13 females.The average age is 33.1±13.8y.Two of them are primary or idiopathic TMA with benign course,excellent responses to PE treatment （100％） and high survival rate.Three of them are （systemic lupus erythematosus,SLE） related TMA with severe kidney damage and need renal replacement therapy.The relief rate of PE treatment and survival rate is 33.3％.There are 17 hematopoietic stem cell transplantation associated TMA patients.Their kidney injuries are mild and they don＇ t need renal replacement therapy.We found a response rate of 41.1％ to PE,of whom 17.6％ attained complete remission.The long prognosis in this group is poor and the survival rate is only 11.8％.Conclusion Responses are excellent with primary or idiopathic TMA while are poor in patients who presented with TMA secondary to underlying disorders.Plasmapheresis is a first-line recommendation for primary TMA.However,it can be concerned as a second-line therapy for patients with secondary TMA if they are resistant to routine treatments.
出处
《中国血液净化》
2014年第2期85-89,共5页
Chinese Journal of Blood Purification
关键词
血浆置换
血栓性微血管病
血栓性血小板减少性紫癜
溶血尿毒综合征
造血干细胞移植
Plasmapheresis
Thrombotic microangiopathy
Thrombotic thrombocytopenic purpura
Hemolytic uremic syndrome
Hematopoietic stem cell transplantation
