摘要
目的探讨儿童急性巨核细胞白血病的临床特征及预后影响因素。方法回顾性分析2011年2月至2017年8月收治的22例急性巨核细胞白血病患儿的临床资料,生存分析采用Kaplan-Meier法,行Log-Rank检验。结果 22例患儿的中位随访时间10.8个月。其中4例患儿诊断后放弃治疗,3例在第1疗程化疗过程中放弃治疗,余15例1、2疗程的完全缓解率为66.7%和93.3%,8例患儿随访期间复发。22例患儿2年总生存率、无事件生存率分别为(36.4±13.7)%和(22.2±10.7)%。单因素分析显示,伴有21三体组的2年总生存率高于不伴有21三体组;染色体数目为49～60条组的2年无事件生存率高于其他数目组;初诊白细胞计数<10×109/L组的2年总生存率及无事件生存率高于≥10×109/L组;第1次骨髓完全缓解后行异基因造血干细胞移植组患儿的2年总生存率及无事件生存率高于单纯化疗组,差异均有统计学意义(P<0.05)。结论儿童急性巨核细胞白血病是一类具有高度异质性的疾病,复发率高,预后差。初诊白细胞计数及染色体核型是影响预后的重要因素,骨髓完全缓解后应尽快行异基因造血干细胞移植。
ObjectiveTo explore the clinical manifestations and prognosis of pediatric acute megakaryoblastic leukemia （AMKL）. MethodsThe clinical data of 22 patients admitted for AMKL from February 2011 to August 2017 was retrospectively analyzed. Survival analysis were estimated by Kaplan-Meier method and Log-Rank test. ResultsThe median follow-up time of the 22 patients was 10.8 months, in which 4 cases gave up after diagnosis and 3 cases abandoned treatment in the frst course of induction chemotherapy. The total complete remission （CR） rate of the remaining 15 patients was 66.7% and 93.3% after the frst and second chemotherapy course, respectively. Marrow relapses occurred in 8 patients, with the 2-year OS and EFS rates of 36.4%±13.7% and 22.2%±10.7% , respectively. Univariate analysis showed that the 2-year OS rate of patients with acquired trisomy 21 was signifcantly higher than that in the other patients （P=0.019） and the 2-year EFS rate of patients with chromosome number between 49 to 60 was signifcantly higher than that in the others （P=0.028）. The 2-year OS and EFS rates of patients with initial white blood cell count below 10×109/L were higher than those above 10×109/L （P=0.047, P=0.04） and the 2-year OS and EFS rates of patients who received allogeneic hematopoietic stem cell transplantation （HSCT） in frst CR were higher than those receiving chemotherapy alone （P〈0.05）. ConclusionsAMKL is a highly heterogeneous disease, with a high recurrence rate and a poor prognosis. Initial white blood cell count and karyotype are important factors affecting the long-term therapeutic effect. HSCT should be applied as soon as possible after CR.
作者
薛玉娟
于水
陆爱东
吴珺
左英熹
贾月萍
张乐萍
XUE Yujuan;YU Shui;LU Aidong;WU Jun;ZUO Yingxi;JIA Yueping;ZHANG Leping(Department of Pediatrics,Peking University People＇s Hospital,Beijing 100044,China)
出处
《临床儿科杂志》
CAS
CSCD
北大核心
2018年第11期834-839,共6页
Journal of Clinical Pediatrics
关键词
急性巨核细胞白血病
预后
儿童
acute megakaryoblastic leukemia
prognosis
pediatrics
