新疆地区28例儿童及青少年淋巴母细胞淋巴瘤临床及预后分析

Clinical characteristics and prognosis of 28 kids and adolescents with lymphoblastic lymphoma in Xinjiang Region

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。

Objective To investigate the clinicopathological characteristics and the prognosis of kids and adolescents 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 protocol, 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 patients 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. Patients 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 improve the efficacy and survival of LBL.