ETP-ALL实验室诊断和治疗新进展  

Research progress on laboratory diagnosis and treatment of ETP-ALL

作　　者：刘梦娜 王洪玲 白萍 蔡宇 李莉[1] LIU Mengna;WANG Hongling;BAI Ping;CAI Yu;LI Li(Department of Clinical Laboratory,Shanghai General Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200080,China;Department of Hematology,Shanghai General Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200080,China)

机构地区：[1]上海交通大学医学院附属第一人民医院检验医学中心,上海200080 [2]上海交通大学医学院附属第一人民医院血液科,上海200080

出　　处：《检验医学》2025年第1期1-7,M0004,共8页Laboratory Medicine

摘　　要：早期前体T淋巴细胞(ETP)是起源于骨髓并定植于胸腺的最早的祖细胞,急性早期前体T淋巴细胞白血病(ETP-ALL)是因研究技术的进步被发现的急性T淋巴细胞白血病(T-ALL)的新亚型。传统的血细胞发育观认为,造血细胞遵循从多能干细胞到谱系限制的祖细胞谱系发育的基本过程,造血过程中的第1个谱系发育过程分为共同的髓系和淋巴系,T淋巴细胞谱系限制的最后步骤发生在胸腺。然而,ETP-ALL既具有T细胞和B细胞的分化潜能,也具有髓系细胞分化潜能,这对传统的造血谱系发育规律提出了挑战。ETP-ALL的谱系来源和分化潜能尚不明确,其免疫表型、基因特征,以及诊断和治疗均处于探索阶段,增加了诊断和鉴别诊断的难度。目前,基于流式细胞免疫分型技术的评分系统是诊断ETP-ALL的重要辅助依据。ETP-ALL治疗通常包括诱导治疗、缓解后的巩固强化治疗和维持治疗,但患者对常规化疗方案反应较差,缓解后复发率较高。完全缓解后,符合条件的患者宜尽早进行异基因造血干细胞(HSC)移植。文章就ETP-ALL的起源和调控,以及ETP-ALL实验室诊断和临床治疗的新进展进行综述,以期为ETP-ALL的诊断和治疗提供帮助。Early T-cell precursors(ETP)are the earliest progenitors that originate from the bone marrow and reside in the thymus.Early T-cell precursor acute lymphoblastic leukemia(ETP-ALL)is a new subtype of T-cell acute lymphoblastic leukemia(T-ALL)that is discovered with the advancement of research techniques.The traditional view of hematopoiesis is that hematopoietic cells follow a basic process of lineage commitment from multipotent stem cells to lineage-restricted progenitor cells.The first step of hematopoiesis is common myeloid and lymphoid differentiation pathways,and the final step of T lymphocyte lineage restriction occurs in the thymus.However,ETP-ALL has differential potential of T,B and myeloid cells,challenging the traditional hematopoietic lineage development rules.At present,the lineage origin and differential potential of ETP-ALL can not be clearly identified,so its immune phenotype,genetic characteristics and diagnosis and treatment are being investigated,which also increases the difficulty of diagnosis and differential diagnosis.Currently,the flow cytometry immunophenotyping scoring system is an important auxiliary diagnostic method of ETP-ALL.ETP-ALL treatment typically includes induction therapy,consolidation therapy after remission and maintenance therapy.Due to poor response to conventional chemotherapy and high relapse rate after remission,patients who achieve complete remission and meet the criteria should perform allogeneic hematopoietic stem cell transplantation as soon as possible.This review provides an overview of the origin and regulation of ETP-ALL,as well as the latest advances in the laboratory diagnosis and clinical treatment of ETP-ALL,which may be useful for its diagnosis and treatment.

关 键 词：急性早期前体T淋巴细胞白血病 早期前体T淋巴细胞 混合表型急性白血病 

分 类 号：R733.71[医药卫生—肿瘤]