急性髓系白血病合并阵发性睡眠性血红蛋白尿患者的临床特征及预后分析  

The Clinical Characteristics and Prognosis of Patients with Acute Myeloid Leukemia Combine Paroxysmal Nocturnal Hemoglobinuria

作　　者：温静 王浩[1] 谢佳[1] 李光 李真真[1] 张晓波 史瑞 宋艳萍 WEN Jing;WANG Hao;XIE Jia;LI Guang;LI Zhen-Zhen;ZHANG Xiao-Bo;SHI Rui;SONG Yan-Ping(Department of Hematology,Xi'an Central Hospital Affiliated to Xi'an Jiaotong University,Xi'an 710003,Shaanxi Province,China)

机构地区：[1]西安交通大学附属西安市中心医院血液内科,陕西西安710003

出　　处：《中国实验血液学杂志》2021年第4期1080-1084,共5页Journal of Experimental Hematology

摘　　要：目的:探讨成人急性髓系白血病(AML)合并阵发性睡眠性血红蛋白尿(PNH)患者的临床特征及预后价值。方法:收集和回顾性分析2017年1月至2019年5月就诊于本院血液内科并接受规律化疗的13例PNH克隆阳性的AML患者的临床资料。分析诱导化疗后完全缓解(CR)率;使用配对t检验对化疗前后PNH病态细胞水平进行检验;采用Kaplan-Meier法及多因素Cox回归模型分析影响预后的因素。结果:13例患者中,11例初次诱导化疗后达CR,CR率为84.6%。中位总生存期(OS)为17(0-30)个月,中位无进展生存期(PFS)为16(2-26)个月。13例患者治疗前后PNH病态细胞变化无统计学差异(P> 0.05)。多因素Cox回归分析显示,患者的年龄、性别、初诊时血红蛋白、血小板数与OS无关(P> 0.05);初诊时白细胞水平、乳酸脱氢酶水平及危险度分层对OS存在影响(P <0.05)。Kaplan-Meier生存分析显示,初诊时白细胞水平小于10×10^(9)/L的AML合并PNH患者OS率明显高于白细胞大于10×10^(9)/L (P=0.0261)的患者。低、中危较高危患者生存率高(P=0.0010)。结论:AML合并PNH患者初次诱导化疗后疗效较好,初诊时白细胞数量及乳酸脱氢酶水平对AML合并PNH患者OS有影响,初诊时白细胞低于10×10^(9)/L及低、中危患者OS率更高。Objective: To investigate the clinical characteristics and prognosis of patients with acute myeloid leukemia( AML) combined with paroxysmal nocturnal hemoglobinuria( PNH). Methods: The clinical data of 13 AML combined with PNH patients treated in our hospital from January 2017 to May 2019 were collected and retrospective analyzed. The complete remission( CR) rate for induction chemotherapy was analyzed. The level of PNH+ cell before and after chemotherapy were tested by Paired t test. Kaplan-Meier method and multi-factorial Cox regression model were used to analyze the influencing factors of prognosis. Results: Among the 13 patients,11( 84. 6%) cases were CR after first induction chemotherapy. The median overall survival( OS) time was 17 months( 0-30 months),the median progression-free survival( PFS) time was 16 months( 2-26 months). There were no significant difference in the number of PNH+ cell before and after chemotherapy( P > 0. 05). Multivariate Cox regression analysis showed that age, sex, the level of hemoglobin,platelet were not related to the OS of the patients( P > 0. 05),the level of WBC,LDH and risk stratification at first diagnosed were related to the OS of the patients( P < 0. 05). Kaplan-Meier survival analysis showed that the OS rate of AML combined with PNH patients with leukocyte lower than 10 × 10^(9)/L at first diagnosed was better than that of the patients with leukocyte higher than 10 × 10^(9)/L( P = 0. 0261). The OS rate of patients with low or standard risk was better than the patients with high risk group( P = 0. 0010) Conclusion: The patients of AML combined with PNH have higher CR rate after the first induction chemotherapy. The level of WBC and LDH at first diagnosed are the factors that affecting the OS of the patients. The OS of patients with WBC lower than 10 × 10^(9)/L,at first diagnosed low and medium risk are better than the other patients.

关 键 词：急性髓系白血病 阵发性睡眠性血红蛋白尿 临床特征 预后 

分 类 号：R733.71[医药卫生—肿瘤] R556.64[医药卫生—临床医学]