CAJ | 학술논문

目的：探讨新疆地区汉族、维吾尔族儿童及青少年淋巴母细胞淋巴瘤（lymphoblastic lymphoma，LBL）患者的临床病理特征及预后。方法回顾性分析新疆医科大学附属肿瘤医院2003年1月-2011年12月收治的28例汉族、维吾尔族儿童及青少年 LBL 患者的临床病理资料，患者的治疗方案包括 CHOP、CHOP 样方案、改良的Hyper-CVAD 和 BFM 方案及自体干细胞移植（autologous stem cell transplantation，ASCT）。采用 Kaplan-Meier法对各方案治疗的患者进行生存分析。结果28例患者中位随访15个月（4～86个月），完全缓解（complete remission，CR）11例，部分缓解（partial remission，PR）2例，疾病进展（progression of disease，PD）15例，总有效率为46．4％。生存13例，死亡15例，死亡的原因主要为疾病进展或复发（1例死于感染）。1年、2年的总体生存率（overall survival，OS）分别为61％、46％。采用改良的 Hyper-CVAD 和 BFM 方案治疗的患者的 OS 显著高于单纯采用 CHOP、CHOP 样方案治疗的患者的 OS （P ＜0．01）。结论新疆地区儿童及青少年 LBL 绝大多数为T-LBL，LBL 总体生存率较低，采用改良的 BFM 方案和 Hyper-CVAD 方案可改善儿童及青少年 LBL 患者预后。
목적：탐토신강지구한족、유오이족인동급청소년림파모세포림파류（lymphoblastic lymphoma，LBL）환자적림상병리특정급예후。방법회고성분석신강의과대학부속종류의원2003년1월-2011년12월수치적28례한족、유오이족인동급청소년 LBL 환자적림상병리자료，환자적치료방안포괄 CHOP、CHOP 양방안、개량적Hyper-CVAD 화 BFM 방안급자체간세포이식（autologous stem cell transplantation，ASCT）。채용 Kaplan-Meier법대각방안치료적환자진행생존분석。결과28례환자중위수방15개월（4～86개월），완전완해（complete remission，CR）11례，부분완해（partial remission，PR）2례，질병진전（progression of disease，PD）15례，총유효솔위46．4％。생존13례，사망15례，사망적원인주요위질병진전혹복발（1례사우감염）。1년、2년적총체생존솔（overall survival，OS）분별위61％、46％。채용개량적 Hyper-CVAD 화 BFM 방안치료적환자적 OS 현저고우단순채용 CHOP、CHOP 양방안치료적환자적 OS （P ＜0．01）。결론신강지구인동급청소년 LBL 절대다수위T-LBL，LBL 총체생존솔교저，채용개량적 BFM 방안화 Hyper-CVAD 방안가개선인동급청소년 LBL 환자예후。
Objective To investigate the clinicopathological characteristics and the prognosis of kids and ad-olescents with lymphoblastic lymphoma (LBL).Methods Retrospective analysis on the records of 28 kids and adolescents with LBL (24 T-LBL and 4 B-LBL)from 2003.1 to 2011.12 in Tumor Hospital affiliated to Xinjiang Medical University was conducted.The patients were treated with CHOP,modified BFM-90 pro-tocol,Hyper-CVAD regimes,and high-dose chemotherapy (HDC)/autologous stem cell transplantation (ASCT).Kaplan-Meier method was adopted for their survival analysis.Results Of the 28 patients,22 pa-tients were male and 6 female.The median age at diagnosis was 10.5 years (4-18 years).Twenty-three cases (82.1%)were in stage Ⅲ or Ⅳ at diagnosis.The response rate (RR)for the whole group was 46.4%,with complete remission (CR)rate of 39.3%.Within a median follow-up duration of 15 months (4-86 months),the 1-year and 2-year overall survival(OS)rates were 61% and 46% respectively.Pa-tients treated with BFM-90,Hyper-CVAD and ASCT had a better prognosis than those treated with only CHOP (P <0.01).Conclusion Lymphoblastic lymphoma in kids and adolescents was highly aggressive with poor prognosis in Xinjiang region.Modified BFM protocol,Hyper-CVAD regimes and ASCT can im-prove the efficacy and survival of LBL.