对2021年7月—2023年7月华中科技大学同济医学院附属同济医院收治的2例PICALM-MLLT10融合基因阳性急性白血病患儿的临床诊疗经过进行回顾性分析。2例患儿分别诊断为急性T淋巴细胞白血病伴中枢神经系统受累和高危急性髓系白血病。2例患儿均在常规化疗联合维奈克拉治疗后达到骨髓完全缓解，基因转阴后序贯异基因造血干细胞移植，随访至今生存状态良好。提示联合维奈克拉化疗后积极行造血干细胞移植，有望提高PICALM-MLLT10阳性白血病患儿的长期生存率。

A retrospective analysis was conducted on the clinical course of two children with PICALM-MLLT10-positive acute leukemia treated at Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, between July 2021 and July 2023. The patients were diagnosed with acute T-lymphoblastic leukemia with central nervous system involvement and high-risk acute myeloid leukemia, respectively. Both achieved bone marrow complete remission after conventional chemotherapy combined with venetoclax. Following conversion to molecular negativity, they underwent sequential allogeneic hematopoietic stem cell transplantation. At the latest follow-up, both patients were alive and in good clinical condition. These observations suggest that proceeding to hematopoietic stem cell transplantation after venetoclax-based chemotherapy may improve the long-term survival of children with PICALM-MLLT10-positive leukemia.