目的　探讨TCF3-PBX1阳性急性淋巴细胞白血病（ALL）的临床特征、疗效及预后影响因素。方法　回顾性 分析2006年8月至2015年8月收治的29例初诊为TCF3-PBX1阳性ALL患儿的临床资料，应用实时定量反转录聚合酶链 式反应定期监测TCF3-PXB1融合基因表达水平，分析影响TCF3-PBX1阳性ALL患儿预后的影响因素。结果　29例患儿， 男16例、女13例，中位年龄8岁（9个月~16岁）。 免疫表型以前B细胞型最多见（58.6%）；染色体核型分析显示以不平衡 易位多见（41.4%）。29例患儿治疗第33天完全缓解率达100%，但微小残留病灶未完全转阴， 3例患儿复发均为微小残留 病（MRD）未转阴者。Cox多因素回归分析显示，年龄是影响5年总生存率（OS）的独立危险因素（P<0.05）。5年OS及无病 生存率（DFS）分别为（82±8）%、（ 81±7）%。结论　伴TCF3-PBX1阳性的儿童ALL是一类具有高度异质性疾病，治疗完 全缓解率高，远期疗效好，对患儿进行危险度分层同时行个体化治疗是提高治愈率的关键。

Objective　To explore the clinical characteristics and relevant factors affecting treatment and prognosis of TCF3-PBX1 positive acute lymphoblastic leukemia (ALL). Methods　The clinical data of 29 children with newly diagnosed TCF3-PBX1 positive ALL from August 2006 to August 2015 were analyzed retrospectively. The expression level of TCF3PXB1 fusion gene was monitored by regular quantitative reverse transcription polymerase chain reaction. The factors influencing prognosis in children with TCF3-PBX1 positive ALL were analyzed. Results　There were 29 children (16 males and 13 females) with a median age of 8 years (9 months to 16 years). The most common immunophenotype was pre-B cell type (pre-B) (58.6%). The karyotype analysis showed that unbalanced translocation was more common (41.4%). The complete remission rate was 100% on thirty-third day in 29 children, but the minimal residual disease (MRD) was not completely negative. Three cases were relapsed, all of whom were MRD positive. Cox multivariate regression analysis showed that age was an independent risk factor for 5 year overall survival (P<0.05). The 5 year overall survival rate and disease-free survival rate were (82±8)% and (81±7)% respectively. Conclusions　Childhood TCF3-PBX1 positive ALL is a highly heterogeneous disease with high rate of complete remission and good long-term efficacy. The risk stratification and individualized treatment is the key to improve the cure rate.