59例血栓性血小板减少性紫癜患者临床分析

Clinical analysis of 59 patients with thrombotic thrombocytopenic purpura

目的对血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)患者的临床特征、诊断、治疗及转归进行分析总结,提高临床诊治水平。方法回顾性分析北京大学人民医院2004年1月至2018年10月收治的59例TTP患者的临床特征及实验室检查,包括血常规、血液生化、免疫学、溶血、血管性血友病因子裂解酶(ADAMTS13)等检查。纳入标准为临床符合TTP诊断,具有三联征或五联征,部分患者检测ADAMTS13活性。依据预后存活与否分组,比较两组间差异。结果59例TTP患者中,男性21例,女性38例,平均年龄46.8岁,55例表现为三联征,46例表现为五联征;血小板、血红蛋白明显减低,外周血破碎红细胞升高,ADAMTS13值或活性明显降低,PLASMIC评分57例为6~7分;59例患者均使用糖皮质激素,41例行血浆置换(plasma exchange,PEX),28例存活;18例未行PEX,6例存活,两者差异有统计学意义(P<0.05)。6例采用利妥昔单抗治疗者,4例存活。结论PLASMIC评分可以较好地预测TTP患者的ADAMTS13活性;PEX能提高TTP患者存活率。

Objective To investigate the clinical features,diagnosis,treatment and prognosis of 59 patients with thrombotic thrombocytopenic purpura(TTP),therefore to improve the ability of diagnosis and treatment.Methods The clinical data of 59 patients with TTP admitted to Peking University People's Hospital from January 2004 to October 2018 were retrospectively analyzed.All the patients were clinically diagnosed,fulfilled the triad syndrome,or quinary syndrome.Laboratory data included complete blood count,blood biochemistry,immunology,hemolysis;some patients tested the activity of ADAMTS13.The differences between groups were compared according to the prognosis.Results Among the 59 patients with TTP,21 were male and 38 were female,with an average age of 46.8 years.Fifty-five patients had the triad syndrome and 46 patients had the quinary syndrome.The platelet count and hemoglobin decreased,the percentage of erythrocyte fragmented increased,and the value or the activity of ADAMTS13 was decreased significantly.PLASMIC scores of 57 patients were between 6 and 7.All 59 patients were treated with glucocorticoid,41 patients received plasma exchange(PEX),and 28 patients survived;18 patients did not received PEX,and only 6 patients survived.There was a significant difference of the survival between the two groups(P<0.05).Six patients were treated with rituximab and four patients survived.Conclusion The PLASMIC score can predict the activity of ADAMTS13 well.PEX can significantly improve the survival rate of patients with TTP.