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中国循证儿科杂志

中国循证儿科杂志 ›› 2020, Vol. 15 ›› Issue (2): 140-144.

• 论著 • 上一篇    下一篇

伴-5/5q-染色体异常的儿童急性髓系白血病12例病例系列报告

郑方圆1，赖悦云2，陆爱东1，左英熹1，贾月萍1，吴珺1，张乐萍1   

  1. 北京大学人民医院 北京，100044；1 儿科，2 血液科
  • 收稿日期:2020-02-06 修回日期:2020-04-24 出版日期:2020-04-25 发布日期:2020-04-25
  • 通讯作者: 张乐萍

Clinical analysis of 12 cases of pediatric acute myeloid leukemia with -5/5q-abnormalities

ZHENG Fang-yuan1, LAI Yue-yun2, LU Ai-dong1, ZUO Ying-xi1, JIA Yue-ping1, WU jun1, ZHANG Le-ping1   

  1. Peking University People's Hospital, Beijing 100044, China; 1 Department of Pediatrics,  2  Department of Hematology
  • Received:2020-02-06 Revised:2020-04-24 Online:2020-04-25 Published:2020-04-25
  • Contact: Zhang Le-ping

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摘要： 目的 探讨-5/5q-染色体异常的儿童急性髓系白血病（AML）的临床特征和预后。方法 纳入2007年1月1日至2018年12月31日北京大学人民医院儿科收治的非复发AML患儿，分析-5/5q-AML患儿的临床特征、实验室检查、治疗方案和预后。结果 584例非复发AML患儿中检出12例（2.05%）-5/5q-，男4例，中位年龄7.5岁，中位随访时间28个月。-5/5q-AML患儿中，3例（25.0%）由MDS转化而来，显著高于无-5/5q-AML患儿（14例，2.4%），P=0.004。-5/5q-与无-5/5q AML患儿在性别构成、发病年龄、初诊时血常规、髓外浸润情况（肝、脾、淋巴结肿大）、诊断前有症状时间、诱导化疗结束后骨髓抑制解除时间方面，差异均无统计学意义。-5/5q-AML患儿诱导化疗采用DAH或ADE方案，7例（583%）达完全缓解，2例（16.7%）达部分缓解，3例（25.0%）未缓解。随访至2019年7月1日，1例失访，6例死亡，5例存活。-5/5q-AML患儿的生存时间为（23.3±26.0）个月，显著短于无-5/5q-AML患儿的生存时间（63.0±10.5）个月，P=0023。-5/5q-AML患儿1年累积生存率和2年累积生存率分别为61.9%和30.9%，显著低于无-5/5q-AML患儿相应的81.6%和77.0%（P＜0.001）。结论 儿童AML中-5/5q-染色体异常的检出率较低，-5/5q-AML患儿中由MDS转化而来者比例高，预后差。

Abstract: Objective To explore the clinical characteristics and prognosis of acute myeloid leukemia (AML) in children with -5/5q-abnormalities. Methods A retrospective analysis was performed for the data of acute myeloid leukemia diagnosed and treated in Department of Pediatrics of Peking University People's Hospital from January 1, 2007 to December 31, 2018, and the clinical manifestations, lab results, treatment and prognosis of children with -5/5q-AML were analyzed. Results Data of 584 children with non-recurrent AML were analyzed in this study. Among them, 12 children(2.05%) were detected with -5/5q-abnormalities, including 4 males, with a median age of 7.5 years and a median follow-up time of 28 months. Three(25%) of the 12 children with -5/5q-AML were transformed from MDS, which was significantly higher than that in children without-5/5q-AML(14/572, 2.4%, P=0.004). There was no significant difference between children with -5/5q-AML and children without -5/5q-AML in terms of gender composition, age at diagnosis, blood routine examination at diagnosis, extramedullary infiltration (hepatomegaly, splenomegaly and lymphnode enlargement), time of symptoms before diagnosis and time of release of myelosuppression after induction chemotherapy. Induction chemotherapy was performed with DAH or ADE regimen in the 12 children with -5/5q-AML. Seven children (58.3%) achieved complete remission; 2 children (16.7%) achieved partial remission; 3 children (25.0%) had no remission. During the follow-up time until July 1, 2019, 1 child lost follow-up; 6 children died ; 5 children survived. The survival time of children with -5/5q-AML (23.3±26.0 months) was significantly shorter than that of children without -5/5q-AML (63.0±10.5 months, P=0.023). The 1-year and 2-year accumulative survival rates of children with -5/5q-AML were 61.9% and 30.9% respectively, which were significantly lower than 81.6% and 77.0% of children without -5/5q-AML, respectively (P＜0.001). Conclusion The detection rate of -5/5q-abnormalities in children with AML was low and the prognosis was poor. The proportion of -5/5q-AML transformed from MDS was high.