伴t(1;19)(E2A-PBX1)的成人急性淋巴细胞白血病的临床特点及疗效分析
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摘要
目的:探讨伴t(1;19)(E2A-PBX1)成人急性淋巴细胞白血病(ALL)的临床特点及疗效。方法:收集2010年11月22日至2018年4月11日我中心收治的19例伴t(1;19)(E2A-PBX1)初治ALL患者的临床资料,分析患者的临床资料特点、化疗及化疗+造血干细胞移植的完全缓解率(CR)、总生存(OS)和无复发生存(RFS)。结果:19例患者中位年龄24岁(14-66岁),男女比例为0.73∶1。中位白细胞数16.47×10~9(1.8-170.34)/L,中位血红蛋白98(65-176)g/L,中位血小板数50(15-254)×10~9/L。免疫分型Pre B-ALL 17例(89.5%),Common B-ALL 2例(10.5%)。总体CR率94.7%,1个疗程CR率94.7%, 4年总生存率47.1%,4年无复发生存率43.3%。造血干细胞移植组患者总生存及无复发生存略高于非移植组患者OS(62.5%vs 36.7%)(P=0.188);RFS(62.5%vs 38.9%)(P=0.166)。结论:成人伴t(1;19)(E2A-PBX1)阳性的ALL患者主要表现为Pre B-ALL,与儿童同类患者疗效比较,疗效较差,造血干细胞移植可提高患者疗效。
Objective: To explore the clinical features and therapeutic efficacy in adult ALL patients with t(1; 19)(E2 A-PBX1). Methods: The clinic data of 19 adult ALL patients with t(1;19)(E2 A-PBX1) in our hospital from Nov. 22,2010 to Apr. 4, 2018 were collected. The clinical features,complete remission(CR) rate, overall survival(OS) rate and relapse-free survival(RFS) rate of patients received chemotherapy and chemotherapy+HSCT were analyzed. Results:In all the 19 patients, the median age was 24(14-66), median WBC count was 16.47 x 109(1.8-170.34)/L, median Hb level was 98(65-176) g/L, median Plt count was 50(15-254) x 109/L. Pre B-ALL were 17 cases(89.5%), and common B-ALL were 2 cases(10.5%). Patients received the induction therapy, the overall CR rate was 94.7%, one course CR rate was 94.7%, 4 year OS rate was 47.1% and RFS rate was 43.3%. The OS rate and RFS rate of patients received transplantation were slightly higher than those of patients not received transplantation(OS: 62.5% vs 36.7%)(P=0.188);RFS(62.5%vs 38.9%)(P=0.166). Conclusion: Most adult ALL patients with t(1;19)(E2 A-PBX1) is Pre B-ALL by Immunophenotyping, as compared with the pediatric patients, the therapeutic efficacy for adult patients with t(1;19)(E2 A-PBX1) is worsen, therefore, stem cell transplantation is still acquired for better long term survival.
Objective: To explore the clinical features and therapeutic efficacy in adult ALL patients with t(1; 19)(E2 A-PBX1). Methods: The clinic data of 19 adult ALL patients with t(1;19)(E2 A-PBX1) in our hospital from Nov. 22,2010 to Apr. 4, 2018 were collected. The clinical features,complete remission(CR) rate, overall survival(OS) rate and relapse-free survival(RFS) rate of patients received chemotherapy and chemotherapy+HSCT were analyzed. Results:In all the 19 patients, the median age was 24(14-66), median WBC count was 16.47 x 109(1.8-170.34)/L, median Hb level was 98(65-176) g/L, median Plt count was 50(15-254) x 109/L. Pre B-ALL were 17 cases(89.5%), and common B-ALL were 2 cases(10.5%). Patients received the induction therapy, the overall CR rate was 94.7%, one course CR rate was 94.7%, 4 year OS rate was 47.1% and RFS rate was 43.3%. The OS rate and RFS rate of patients received transplantation were slightly higher than those of patients not received transplantation(OS: 62.5% vs 36.7%)(P=0.188);RFS(62.5%vs 38.9%)(P=0.166). Conclusion: Most adult ALL patients with t(1;19)(E2 A-PBX1) is Pre B-ALL by Immunophenotyping, as compared with the pediatric patients, the therapeutic efficacy for adult patients with t(1;19)(E2 A-PBX1) is worsen, therefore, stem cell transplantation is still acquired for better long term survival.
引文
1 Faderl S,Jeha S,Kantarjian HM.The biology and therapy of adult acute lymphoblastic leukemia.Cancer,2003;98(7):1337-1354.
2 Pui CH,Evans WE.Acute lymphoblastic leukemia.N Engl J Med,1998;339(9):605-615.
3 Dhe din N,Huynh A,Maury S,et al.Role of allogeneic stem cell transplantation in adult patients with Ph-negative acute lymphoblastic leukemia.Blood,2015;125(16):2486-2496.
4赵邢力,魏辉,林冬,等.成人Ph阴性急性淋巴细胞白血病的优化治疗.中华血液学杂志,2014;35(10):873-879.
5 Siegel SE,Advani A,Seibel N,et al.Treatment of young adults with Philadelphia-negative acute lymphoblastic leukemia and lymphoblastic lymphoma:Hyper-CVAD vs.pediatric-inspired regimens.Am J Hematol,2018;93(10):1254-1266.
6 Fière D,Lepage E,Sebban C,et al.Adult acute lymphoblastic leukemia:a multicentric randomized trial testing bone marrow transplantation as post-remission therapy.The French Group on Therapy for Adult Acute Lymphoblastic Leukemia.J Clin Oncol,1993;11(10):1990-2001.
7 Gupta V,Richards S,Rowe J.Allogeneic,but not autologous,hematopoietic cell transplantation improves survival only among younger adults with acute lymphoblastic leukemia in first remission:An individual patient data meta-analysis.Blood,2013;121(2):339-350.
8 G?kbuget N,Hoelzer D,Arnold R,et al.Treatment of Adult ALLaccording to protocols of the German Multicenter Study Group for Adult ALL(GMALL).Hematol Oncol Clin North Am,2000;14(6):1307-1325.
9 Huguet F,Chevret S,Leguay T,et al.Intensified therapy of acute lymphoblastic leukemia in adults:report of the randomized GRAALL-2005 clinical trial.J Clin Oncol,2018;36(24):2514-2523.
10 DeAngelo DJ,Stevenson KE,Dahlberg SE,et al.Long-term outcome of a pediatric-inspiredregimen used for adults aged 18-50 years with newly diagnosed acute lymphoblastic leukemia.Leukemia,2015;29(3):526-534.
11 Vey N,Thomas X,Picard C,et al.Allogeneic stem cell transplantation improves the outcome of adults with t(1;19)/E2A-PBX1 and t(4;11)/MLL-AF4 positive B-cell acute lymphoblastic leukemia:results of the prospective multicenter LALA-94 study.Leukemia,2006;20(12):2155-2161.
12 Hu YX,He HL,Lu J,et al.E2A-PBX1 exhibited a promising prognosis in pediatric acute lymphoblastic leukemia treated with the CCLG-ALL-2008 protocol.OncoTargets and Therapy,2016:97219-7225.
13 Hu YX,Lu J,He HL,et al.A prospective evaluation of minimal residual disease as risk stratification for CCLG-ALL-2008 treatment protocol in pediatric B precursor acute lymphoblastic leukemia.Eur Rev Med Pharmacol Sci,2016;May;20(9):1680-1690.
14 Bostrom BC,Sensel MR,Sather HN,et al.Dexamethasone versus prednisone and daily oral versus weekly intravenous mercaptopurine for patients with standard risk acute lymphoblastic leukemia:a report from the Children's Cancer Group.Blood,2003;101(10):3809-3817.
15 Schwartz CL,Thompson EB,Gelber RD,et al.Improved response with higher corticosteroid dose in children with acute lymphoblastic leukemia.J Clin Oncol,2001;19(4):1040-1046.
16 Eden OB,Stiller CA,Gerrard MP,et al.Improved survival for childhood acute lymphoblastic leukemia:possible effect of protocol compliance.Pediatr Hematol Oncol,1988;5(2):83-91.
17 Larsen EC,Devidas M,Chen S,et al.Dexamethasone and high-dose methotrexate improve outcome for children and young adults with high-risk B-acute lymphoblastic leukemia:A report from Children's Oncology Group Study AALL0232.J Clin Oncol,2016;34(20):2380-2388.
18 Stock W,La M,Sanford B,et al.What determines the outcomes for adolescents and young adults with acute lymphoblastic leukemia treated on cooperative group protocols?A comparison of Children's Cancer Group and Cancer and Leukemia Group B studies.Blood,2008;112(5):1646-1654.
2 Pui CH,Evans WE.Acute lymphoblastic leukemia.N Engl J Med,1998;339(9):605-615.
3 Dhe din N,Huynh A,Maury S,et al.Role of allogeneic stem cell transplantation in adult patients with Ph-negative acute lymphoblastic leukemia.Blood,2015;125(16):2486-2496.
4赵邢力,魏辉,林冬,等.成人Ph阴性急性淋巴细胞白血病的优化治疗.中华血液学杂志,2014;35(10):873-879.
5 Siegel SE,Advani A,Seibel N,et al.Treatment of young adults with Philadelphia-negative acute lymphoblastic leukemia and lymphoblastic lymphoma:Hyper-CVAD vs.pediatric-inspired regimens.Am J Hematol,2018;93(10):1254-1266.
6 Fière D,Lepage E,Sebban C,et al.Adult acute lymphoblastic leukemia:a multicentric randomized trial testing bone marrow transplantation as post-remission therapy.The French Group on Therapy for Adult Acute Lymphoblastic Leukemia.J Clin Oncol,1993;11(10):1990-2001.
7 Gupta V,Richards S,Rowe J.Allogeneic,but not autologous,hematopoietic cell transplantation improves survival only among younger adults with acute lymphoblastic leukemia in first remission:An individual patient data meta-analysis.Blood,2013;121(2):339-350.
8 G?kbuget N,Hoelzer D,Arnold R,et al.Treatment of Adult ALLaccording to protocols of the German Multicenter Study Group for Adult ALL(GMALL).Hematol Oncol Clin North Am,2000;14(6):1307-1325.
9 Huguet F,Chevret S,Leguay T,et al.Intensified therapy of acute lymphoblastic leukemia in adults:report of the randomized GRAALL-2005 clinical trial.J Clin Oncol,2018;36(24):2514-2523.
10 DeAngelo DJ,Stevenson KE,Dahlberg SE,et al.Long-term outcome of a pediatric-inspiredregimen used for adults aged 18-50 years with newly diagnosed acute lymphoblastic leukemia.Leukemia,2015;29(3):526-534.
11 Vey N,Thomas X,Picard C,et al.Allogeneic stem cell transplantation improves the outcome of adults with t(1;19)/E2A-PBX1 and t(4;11)/MLL-AF4 positive B-cell acute lymphoblastic leukemia:results of the prospective multicenter LALA-94 study.Leukemia,2006;20(12):2155-2161.
12 Hu YX,He HL,Lu J,et al.E2A-PBX1 exhibited a promising prognosis in pediatric acute lymphoblastic leukemia treated with the CCLG-ALL-2008 protocol.OncoTargets and Therapy,2016:97219-7225.
13 Hu YX,Lu J,He HL,et al.A prospective evaluation of minimal residual disease as risk stratification for CCLG-ALL-2008 treatment protocol in pediatric B precursor acute lymphoblastic leukemia.Eur Rev Med Pharmacol Sci,2016;May;20(9):1680-1690.
14 Bostrom BC,Sensel MR,Sather HN,et al.Dexamethasone versus prednisone and daily oral versus weekly intravenous mercaptopurine for patients with standard risk acute lymphoblastic leukemia:a report from the Children's Cancer Group.Blood,2003;101(10):3809-3817.
15 Schwartz CL,Thompson EB,Gelber RD,et al.Improved response with higher corticosteroid dose in children with acute lymphoblastic leukemia.J Clin Oncol,2001;19(4):1040-1046.
16 Eden OB,Stiller CA,Gerrard MP,et al.Improved survival for childhood acute lymphoblastic leukemia:possible effect of protocol compliance.Pediatr Hematol Oncol,1988;5(2):83-91.
17 Larsen EC,Devidas M,Chen S,et al.Dexamethasone and high-dose methotrexate improve outcome for children and young adults with high-risk B-acute lymphoblastic leukemia:A report from Children's Oncology Group Study AALL0232.J Clin Oncol,2016;34(20):2380-2388.
18 Stock W,La M,Sanford B,et al.What determines the outcomes for adolescents and young adults with acute lymphoblastic leukemia treated on cooperative group protocols?A comparison of Children's Cancer Group and Cancer and Leukemia Group B studies.Blood,2008;112(5):1646-1654.