摘要
目的:总结分析成人早期前体T细胞急性淋巴细胞白血病(early T-cell precursor acute lymphoblastic leukemia,ETP-ALL)的临床特征、治疗效果及长期预后。方法:回顾性研究2020年4月至2023年12月诊治的15例ETP-ALL患者,分析基本临床特征、流式免疫分型、细胞遗传学及分子生物学异常、治疗方案与预后的关系。结果:成人急性T淋巴细胞白血病患者中ETP-ALL的发生率为21.8%,其中男性占73.3%,中位年龄43岁,中位骨髓原始细胞比例71.1%。髓系/干细胞抗原阳性表达CD34、CD117、HLA-DR、CD13、CD33等。22.2%的患者合并染色体异常,80.0%的患者合并分子生物学异常,常见的基因突变包括NOTCH1、PHF6、JAK3、ETV6、JAK1、WT1等。第1个疗程诱导缓解治疗后73.3%的患者达完全缓解,66.7%的患者达微小残留病阴性的完全缓解。中位随访时间15.4个月,1年及2年总生存率分别为90.9%和77.9%,1年及2年无复发生存率分别为86.2%和64.6%。结论:成人ETP-ALL同时具有淋系和髓系的临床特征,在VDCLP化疗方案的基础上联合应用髓系化疗药物或多种靶向药物或可提升完全缓解率,在第1次完全缓解期间尽快行异基因造血干细胞移植或可延长生存期。
Objective: To summarize and analyze the clinical characteristics,treatment outcomes,and long-term prognosis of adult early T-cell precursor acute lymphoblastic leukemia(ETP-ALL).Methods: A retrospective study was conducted on 15 ETP-ALL patients diagnosed and treated from April 2020 to December 2023.The relationships between basic clinical characteristics,immunophenotyping,cytogenetic and molecular biological abnormalities,treatment regimens,and prognosis were analyzed.Results: The incidence of ETP-ALL among adult T-cell acute lymphoblastic leukemia patients was 21.8%,with 73.3%being male,a median age of 43 years,and a median percentage of bone marrow blast cells of 71.1%.Positive expressions of myeloid/stem cell antigens included CD34,CD117,HLA-DR,CD13,CD33,etc.22.2%of patients had chromosomal abnormalities,and 80.0%of patients had molecular biology abnormalities,with common gene mutations including NOTCH1,PHF6,JAK3,ETV6,JAK1,WT1,etc.After the first course of induction therapy,73.3%of patients achieved complete remission,and 66.7%achieved minimal residual disease negative complete remission.The median follow-up time was 15.4 months,with 1-year and 2-year overall survival rates of 90.9%and 77.9%,and 1-year and 2-year relapse-free survival rates of 86.2%and 64.6%,respectively.Conclusion: Adult ETP-ALL presents with both lymphoid and myeloid characteristics.Adding myeloid chemotherapy drugs or multiple targeted therapies to the VDCLP regimen may improve complete remission rates,and performing allogeneic hematopoietic stem cell transplantation as soon as possible during the first complete remission period may prolong the survival.
作者
胡池玥
卢聪
江慧雯
HU Chiyue;LU Cong;JIANG Huiwen(Medical College of Nanchang University,Nanchang,330006,China;Institute of Hematology,Union Hospital,Tongji Medical College,Huazhong University of Science and Technology)
出处
《临床血液学杂志》
CAS
2024年第3期162-167,共6页
Journal of Clinical Hematology
基金
国家自然科学基金(No:82200144)。
关键词
早期前体T细胞
急性淋巴细胞白血病
临床特征
治疗
early T-cell precursors
acute lymphoblastic leukemia
clinical characteristics
treatment
