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T型急性淋巴细胞白血病(T-cell acute lymphoblastic leukemia，T-ALL)是儿童和青少年常见的血液恶性疾病之一，其进展迅速，病死率高。自1991年首次在伴有t(7；9)转位的T-ALL中发现NOTCH1一类活化型突变以来，随后多年对NOTCH1基因突变与T-ALL之间的致病机制和临床治疗均取得一定的进展，但对于儿童和成年人使用化疗药物后的预后仍然有差异。因此，该研究主要对伴有NOTCH1基因突变的急性淋巴细胞白血病成年人和儿童预后的差异及最新治疗进展进行综述，以期为临床治疗提供指导。“,”T-cell acute lymphonlastic leukemia(T-ALL) is one of the common hematological malignancies in children and adolescents, and it has a rapid progress and high mortality.Since the first activated mutation in NOTCH1 was found in T-ALL with t(7; 9) translocation in 1991.The pathogenesis and clinical treatment of NOTCH1 gene mutation and T-ALL had made some progress at subsequent years.However, there are still some differences in the prognosis of children and adults after using chemotherapy drugs.Therefore, the differences of prognosis of acute lymphoblastic leukemia with NOTCH1 gene mutation in adults and children and the latest treatment progress are reviewed and hope to provide guidance for clinical treatment.