Acute lymphoblastic leukemia includes T-cell acute lymphoblastic leukemia(T-ALL)and B-cell acute lymphoblastic leukemia(B-ALL).In children,T-ALL usually has a worse prognosis than B-ALL,although childhood T-ALL progno...Acute lymphoblastic leukemia includes T-cell acute lymphoblastic leukemia(T-ALL)and B-cell acute lymphoblastic leukemia(B-ALL).In children,T-ALL usually has a worse prognosis than B-ALL,although childhood T-ALL prognoses have improved remarkably.The varying outcomes among T-ALL cases suggest that an unrecognized biological heterogeneity may contribute to chemo-resistance.Deep exploration of T-lymphocyte development in recent years has found a subgroup of patients with a phenotype that resembles early T-cell precursor,which confers a much poorer prognosis than any other form of T-ALL.This novel subtype of T-ALL was called early T-cell precursor acute lymphoblastic leukemia(ETP-ALL).Flow cytometry data from T-ALL patients enrolled in Shanghai Children’s Medical Center between July 2002 and October 2010 were assessed according to Dr.Campana’s protocol.Among total 89 T-ALL cases,74 cases had enough immunophenotype data available to differentiate between ETP(CD1a^(-),CD8^(-),CD5^(dim),at least one marker of stem cell or myeloid lineage)and non-ETP.From these 74 subjects,12 ETP-ALL cases(16.2%)were identified.The event-free survival(EFS)rate at 66.8 months was 11.1%±10.1% for ETP-ALL and 57.6%±5.6% for non-ETP-ALL(P=0.003).The overall survival rates were 13.3%±11.0% for ETP-ALL and 64.7%±6.3% for non-ETP-ALL(P=0.002).Our findings demonstrate that early T-cell precursor leukemia is a very high-risk subtype of acute lymphoblastic leukemia with poor prognosis.展开更多
目的:对3个血液学中心的成人早期前体T细胞白血病/淋巴瘤(ETP-ALL/LBL)患者进行回顾性分析,总结其临床特点、治疗及预后影响因素。方法:收集2006年1月至2019年1月来自北京大学第三医院、解放军第一医学中心和中国医学科学院血液学研究所...目的:对3个血液学中心的成人早期前体T细胞白血病/淋巴瘤(ETP-ALL/LBL)患者进行回顾性分析,总结其临床特点、治疗及预后影响因素。方法:收集2006年1月至2019年1月来自北京大学第三医院、解放军第一医学中心和中国医学科学院血液学研究所3个血液研究中心共113例T淋巴母细胞白血病/淋巴瘤(T-ALL/LBL)患者的临床数据资料,对其中ETP-ALL/LBL及非ETP-ALL/LBL患者的临床特征及预后进行分析比较。结果:113例T-ALL/LBL患者中,13例诊断为ETP-ALL/LBL(11.5%),其中男性患者11例(84.6%),中位年龄28(18-53)岁。与非ETP-ALL/LBL患者相比,ETP-ALL/LBL患者在年龄、性别、纵隔大包块发生率、临床分期、IPI评分、白细胞水平、乳酸脱氢酶水平方面差异无统计学意义。在13例ETP-ALL/LBL患者中,9例(69.2%)获得完全缓解,ETP-ALL/LBL患者较非ETP-ALL/LBL患者化疗诱导缓解率无统计学差异。在单纯化疗未进行异基因造血干细胞移植的患者中,ETP-ALL/LBL组较非ETP-ALL/LBL组显示出更差的5年生存率(0 vs 7.1%,P=0.008),而在进行异基因造血干细胞移植的患者中,两组5年生存率无统计学差异(37.5%vs 40.2%,P>0.05)。多因素Cox回归分析提示,诱导治疗达到完全缓解、异基因造血干细胞移植以及乳酸脱氢酶水平为影响T-ALL/LBL的独立预后因素。结论:ETP-ALL/LBL较其他类型T-ALL/LBL患者诱导化疗反应率无显著差异,诱导缓解后续贯异基因造血干细胞移植巩固治疗对于提高ETP-ALL/LBL患者远期生存率具有重要意义。展开更多
基金partly supported by National Natural Science Foundation of China(Grant No.30670883).
文摘Acute lymphoblastic leukemia includes T-cell acute lymphoblastic leukemia(T-ALL)and B-cell acute lymphoblastic leukemia(B-ALL).In children,T-ALL usually has a worse prognosis than B-ALL,although childhood T-ALL prognoses have improved remarkably.The varying outcomes among T-ALL cases suggest that an unrecognized biological heterogeneity may contribute to chemo-resistance.Deep exploration of T-lymphocyte development in recent years has found a subgroup of patients with a phenotype that resembles early T-cell precursor,which confers a much poorer prognosis than any other form of T-ALL.This novel subtype of T-ALL was called early T-cell precursor acute lymphoblastic leukemia(ETP-ALL).Flow cytometry data from T-ALL patients enrolled in Shanghai Children’s Medical Center between July 2002 and October 2010 were assessed according to Dr.Campana’s protocol.Among total 89 T-ALL cases,74 cases had enough immunophenotype data available to differentiate between ETP(CD1a^(-),CD8^(-),CD5^(dim),at least one marker of stem cell or myeloid lineage)and non-ETP.From these 74 subjects,12 ETP-ALL cases(16.2%)were identified.The event-free survival(EFS)rate at 66.8 months was 11.1%±10.1% for ETP-ALL and 57.6%±5.6% for non-ETP-ALL(P=0.003).The overall survival rates were 13.3%±11.0% for ETP-ALL and 64.7%±6.3% for non-ETP-ALL(P=0.002).Our findings demonstrate that early T-cell precursor leukemia is a very high-risk subtype of acute lymphoblastic leukemia with poor prognosis.
文摘目的:对3个血液学中心的成人早期前体T细胞白血病/淋巴瘤(ETP-ALL/LBL)患者进行回顾性分析,总结其临床特点、治疗及预后影响因素。方法:收集2006年1月至2019年1月来自北京大学第三医院、解放军第一医学中心和中国医学科学院血液学研究所3个血液研究中心共113例T淋巴母细胞白血病/淋巴瘤(T-ALL/LBL)患者的临床数据资料,对其中ETP-ALL/LBL及非ETP-ALL/LBL患者的临床特征及预后进行分析比较。结果:113例T-ALL/LBL患者中,13例诊断为ETP-ALL/LBL(11.5%),其中男性患者11例(84.6%),中位年龄28(18-53)岁。与非ETP-ALL/LBL患者相比,ETP-ALL/LBL患者在年龄、性别、纵隔大包块发生率、临床分期、IPI评分、白细胞水平、乳酸脱氢酶水平方面差异无统计学意义。在13例ETP-ALL/LBL患者中,9例(69.2%)获得完全缓解,ETP-ALL/LBL患者较非ETP-ALL/LBL患者化疗诱导缓解率无统计学差异。在单纯化疗未进行异基因造血干细胞移植的患者中,ETP-ALL/LBL组较非ETP-ALL/LBL组显示出更差的5年生存率(0 vs 7.1%,P=0.008),而在进行异基因造血干细胞移植的患者中,两组5年生存率无统计学差异(37.5%vs 40.2%,P>0.05)。多因素Cox回归分析提示,诱导治疗达到完全缓解、异基因造血干细胞移植以及乳酸脱氢酶水平为影响T-ALL/LBL的独立预后因素。结论:ETP-ALL/LBL较其他类型T-ALL/LBL患者诱导化疗反应率无显著差异,诱导缓解后续贯异基因造血干细胞移植巩固治疗对于提高ETP-ALL/LBL患者远期生存率具有重要意义。
