目的 分析伴单体7（-7）和7号染色体长臂缺失（7q-）的儿童急性髓系白血病（acute myeloid leukemia, AML）的临床特征及预后相关因素。 方法 回顾性分析2010年1月—2024年12月北京大学人民医院儿科收治的伴-7/7q- AML儿童的临床资料、治疗及预后。 结果 共收治染色体核型分析结果完整的AML儿童869例，其中伴-7/7q-染色体异常32例（3.7%）；男20例，女12例；中位诊断年龄6岁。伴孤立性-7染色体异常6例（19%），伴孤立性7q-染色体异常2例（6%），伴额外染色体异常24例（75%）。诱导化疗后获得完全缓解16例（50%）。随访时死亡15例（47%），存活17例（53%），3年无病生存率为（54.1±0.1）%，3年总生存率为（52.6±0.1）%，行造血干细胞移植（hematopoietic stem cell transplantation, HSCT）为3年无病生存率（HR=0.17，95%CI：0.04~0.62，P=0.008）及总生存率（HR=0.16，95%CI：0.04~0.59，P=0.006）的独立影响因素，行HSCT治疗的儿童可获得更好的预后。 结论 -7/7q-染色体在儿童AML中的发生率为3.7%，易合并额外染色体异常，诱导化疗后完全缓解率低，行HSCT治疗有助于改善预后，提高生存率。

Objective To explore the clinical characteristics and prognostic factors of pediatric acute myeloid leukemia (AML) with monosomy 7 (-7) and deletion of the long arm of chromosome 7 (7q-). Methods A retrospective study was conducted on the clinical data, treatment, and prognosis of children with -7/7q- AML who were admitted to the Department of Pediatrics at Peking University People's Hospital from January 2010 to December 2024. Results A total of 869 children with AML who had complete karyotype data were included, of whom 32 (3.7%) had -7/7q- chromosomal abnormalities. There were 20 males and 12 females, and the median age at diagnosis was 6 years. Six children (19%) had isolated -7; 2 (6%) had isolated 7q-; and 24 (75%) had additional chromosomal abnormalities. After induction chemotherapy, complete remission (CR) was achieved in 16 children (50%). At the last follow-up, 15 children (47%) had died and 17 (53%) were alive. The 3-year disease-free survival (DFS) rate was (54.1±0.1)%, and the 3-year overall survival (OS) rate was (52.6±0.1)%. The multivariable analysis showed that hematopoietic stem cell transplantation (HSCT) was an independent prognostic factor for DFS (HR=0.17, 95%CI: 0.04-0.62, P=0.008) and OS (HR=0.16, 95%CI: 0.04-0.59, P=0.006), with better outcomes in children who underwent HSCT. Conclusions The incidence of -7/7q- chromosomal abnormalities in children with AML is 3.7%. Additional chromosomal aberrations are common, and the CR rate after induction chemotherapy is low. HSCT is associated with improved prognosis and survival.