血栓性血小板减少性紫癜患者的临床分析  

Clinical analysis of patients with thrombotic thrombocytopenic purpura

作　　者：石茂静[1] 黄文凤[1] 高伟波[1] 朱继红[1] Shi Mao-jing;Huang Wen-feng;Gao Wei-bo;Zhu Ji-hong(Peking University People′s Hospital, Beijing 100044,China)

机构地区：[1]北京大学人民医院急诊科,北京100044

出　　处：《中国急救医学》2019年第7期667-671,共5页Chinese Journal of Critical Care Medicine

摘　　要：目的探讨血栓性血小板减少性紫癜(TTP)的临床特征、诊断、治疗及转归。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患者的临床资料。结果 59例TTP患者中,男性21例,女性38例,平均年龄(46.8±16.2)岁,55例表现为三联征,46例表现为五联征。血小板(13.2±12.0)×10^9/L,血红蛋白(66.8±20.7)g/L,外周血破碎红细胞(4.6±4.1)%,游离血红蛋白(426.2±298.2)mg/L,血管性血友病因子裂解酶(ADAMTS13)值或活性明显降低。59例患者均使用糖皮质激素,41例行血浆置换者,28例存活;18例未行血浆置换者,6例存活,二者差异有统计学意义(P<0.05)。6例用利妥昔单抗治疗者,4例存活。结论及早治疗,使用血浆置换能明显提高TTP患者存活率,对于难治性TTP患者,建议尽早使用利妥昔单抗。Objective To investigate the clinical features, diagnosis, treatment and prognosis of thrombotic thrombocytopenic purpura ( TTP). 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. Results Among 59 patients with TTP, 21 were males and 38 were females, with an average age of (46. 8 ± 16. 2) years. Fifty - five patients had the triad syndrome and 46 patients had the quinary syndrome. The average counts of platelet, hemoglobin, the percentage of erythrocyte fragments and free hemoglobin were (13.2±12.0) x 10^9/L,(66. 8 ± 20. 7) g/L,(4.6 ±4. 1)% and (426. 2 ±298.2) mg/L, respectively. The value or the activity of ADAMTS 13 decreased significantly. Fifty - nine patients were treated with glucocorticoid. Of 41 cases with plasma exchange, 28 cases survived, but of 18 cases without plasma exchange, 6 cases survived, there was a significant difference between the two groups (P <0. 05 ). Six patients were treated with rituximab and four survived. Conclusion If TTP patients cannot be treated as soon as possible, the mortality is high, and plasma exchange can significantly improve the survival rate. Rituximab can be used as early as possible in patients with refractory TTP.

关 键 词：血栓性血小板减少性紫癜(TTP) 血管性血友病因子裂解酶(ADAMTS13) 血浆置换 利妥昔单抗 

分 类 号：R554.6[医药卫生—血液循环系统疾病]