成人早期前体T急性淋巴细胞白血病的临床特征及预后

黄走方; 王婷玉; 傅明伟; 安刚; 邹德慧; 郝牧; 邱录贵

doi:10.13201/j.issn.1004-2806.2018.11.005

成人早期前体T急性淋巴细胞白血病的临床特征及预后

- 实验血液学国家重点实验室北京协和医学院&中国医学科学院血液病研究所血液病医院淋巴瘤中心(天津, 300020)
基金项目:
十二五国家科技支撑计划项目(No:2014BAI0912)

中国医学科学院医学与健康科技创新工程(No:CAMS-2017-I2M-1-005,CAMS-2016-I2M-3-013)

国家自然科学基金(No:81630007,81570181)

详细信息

通讯作者: 邱录贵,E-mail:qiulg@ihcams.ac.cn

中图分类号: R733.7

收稿日期: 2018-09-23

Clinical features and treatment outcome of adult early T cell precursor acute lymphoblastic leukemia

- Department of Lymphoma Center, State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Diseases Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Tianjin, 300020, China

More Information

Corresponding author: QIU Lugui, E-mail: qiulg@ihcams.ac.cn

Received Date: 23 September 2018

摘要

摘要: 目的:探讨成人早期前体T急性淋巴细胞白血病(ETP-ALL)的临床特征与治疗效果。方法:回顾性研究71例成人T细胞急性淋巴细胞白血病(T-ALL)患者,根据免疫表型定义ETP-ALL,分析其临床特征和疗效。结果:71例成人T-ALL中有15例(21.1%)ETP-ALL患者。与其他T-ALL患者相比,ETP-ALL的中位年龄、血小板计数更高(31岁:21岁,P=0.008;102×10⁹/L:55×10⁹/L,P=0.015),而白细胞计数显著更低(8.5×10⁹/L:53×10⁹/L,P=0.008)。二代测序筛查4例患者基因突变,检出JAK3、JAK1、ETV6、DNMT3A、p53等突变,其中3例有JAK3突变。与其他T-ALL患者相比,ETP-ALL患者1个疗程诱导治疗完全缓解率低(46.2%:73.9%,P=0.059),第1个疗程和第2个疗程微小残留病阳性率高(84.6%:50.0%,P=0.026;72.7%:27.9%,P=0.006)。ETP-ALL与其他T-ALL患者的5年无复发生存率及总生存率比较均差异无统计学意义(18%:24%,P=0.471;27%:19%,P=0.515)。结论:成人ETP-ALL早期治疗反应差,MRD阳性率高。
- 淋巴样白血病 /
- T细胞 /
- 治疗
Abstract: Objective:To analyze clinical features and treatment outcome of adult early T cell precursor acute lymphoblastic leukemia (ETP-AL).Method:In this retrospective analysis,we included 71 adult patients with T cell acute lymphoblastic leukemia (T-ALL).We performed clinicobiologic and therapeutic analyses of patients with ETP-ALL defined by immunophenotyping.Result:Fifteen patients (21.1%) had leukemic lymphoblasts with ETP-ALL associated distinctive immunopehnotype.Compared with other T-ALL patients,the median age and platelet count in patients with ETP-ALL were higher (31 years vs 21 years,P=0.008;102×10⁹/L vs 55×10⁹/L,P=0.015),while the WBC count was significantly lower (8.5×10⁹/L vs 53×10⁹/L,P=0.008).With the use of next generation sequencing,JAK3,JAK1,ETV6,DNMT3A,p53 and other mutations were detected in 4 cases with ETP-ALL.Three of the 4 patients had JAK3 gene mutation.The complete remission rate and minimal residual disease (MRD) after the first course of induction treatment were inferior to patients with other T-ALL (46.2% vs 73.9%,P=0.059; 84.6% vs 50.0%,P=0.026),the same results happened as MRD after the second treatment course (72.7% vs 27.9%,P=0.006).The relapse-free survival rate and overall survival rate for adults with ETP-ALL group were not inferior to those of the other T-ALL group (18% vs 24%,P=0.471; 27% vs 19%,P=0.515).Conclusion:Patients with ETP-ALL have poor response to early treatment and high positive rate of MRD.
- lymphoid leukemia /
- T cell /
- treatment

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参考文献(11)

[1]	Iacobucci I,Mullighan CG.Genetic basis of acute lymphoblastic leukemia[J].J Clin Oncol,2017,35:975-983.
[2]	Coustan-Smith E,Mullighan CG,Onciu M,et al.Early T-cell precursor leukaemia:a subtype of very high-risk acute lymphoblastic leukaemia[J].Lancet Oncol,2009,10:147-156.
[3]	Bond J,Graux C,Lhermitte L,et al.Early response-based therapy stratification improves survival in adult early thymic precursor acute lymphoblastic leukemia:A group for research on adult acute lymphoblastic leukemia study[J].J Clin Oncol,2017,35:2683-2691.
[4]	Bell JJ,Bhandoola A.The earliest thymic progenitors for T cells possess myeloid lineage potential[J].Nature,2008,452:764-767.
[5]	Jain N,Lamb AV,O'Brien S,et al.Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults:a high-risk subtype[J].Blood,2016,127:1863-1869.
[6]	Marks DI,Paietta EM,Moorman AV,et al.T-cell acute lymphoblastic leukemia in adults:clinical features,immunophenotype,cytogenetics,and outcome from the large randomized prospective trial (UKALL XII/ECOG 2993)[J].Blood,2009,114:5136-5145.
[7]	Guo RJ,Bahmanyar M,Minden MD,et al.CD33,not early precursor T-cell phenotype,is associated with adverse outcome in adult T-cell acute lymphoblastic leukaemia[J].Br J Haematol,2016,172:823-825.
[8]	Patrick K,Wade R,Goulden N,et al.Outcome for children and young people with early T-cell precursor acute lymphoblastic leukaemia treated on a contemporary protocol,UKALL 2003[J].Br J Haematol,2014,166:421-424.
[9]	Zhang J,Ding L,Holmfeldt L,et al.The genetic basis of early T-cell precursor acute lymphoblastic leukaemia[J].Nature,2012,481:157-163.
[10]	Lu BY,Thanawala SU,Zochowski KC,et al.Decitabine enhances chemosensitivity of early T-cell precursor-acute lymphoblastic leukemia cell lines and patient-derived samples[J].Leuk Lymphoma,2016,57:1938-1941.
[11]	Maude SL,Dolai S,Delgado-Martin C,et al.Efficacy of JAK/STAT pathway inhibition in murine xenograft models of early T-cell precursor (ETP) acute lymphoblastic leukemia[J].Blood,2015,125:1759-1767.