新疆地区43例淋巴母细胞淋巴瘤的病理及临床特征分析

Clinical characteristics and prognosis of 43 patients with lymphoblastic lymphoma in Xinjiang region of China

目的探讨新疆地区汉族和维吾尔族淋巴母细胞淋巴瘤（lymphoblastic lymphoma，LBL）患者的病理及临床特征。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43例汉族和维吾尔族I。BI．患者的病理及临床资料，治疗方案包括CHOP、CHOP样方案、改良的Hyper—CVAD、BFM方案及自体干细胞移植（autologous stem cell transplantation，ASCT）。采用Kaplan-Meier法进行生存分析，Cox比例风险模型分析影响预后的因素。结果全组患者中位随访时间11个月（4～66个月），CR15例，PR9例，PD19例。生存22例，死亡21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年的总体生存率分别为55％、45％。单因素分析显示，体力状态（performance status，PS）评分、B症状、大纵隔或大肿块、骨髓受侵、脑脊液异常、LDH水平、治疗方式及疗效与LBL患者的预后相关（P＜0．05）。多因素分析显示，LDH水平、治疗方式的选择及疗效评价是新疆地区汉族及维吾尔族LBL患者的独立预后因素（P＜0．05）。结论新疆地区LBL多发生于儿童与青少年，绝大多数为TLBL，总体生存率较低，采用改良的BFM方案或Hyper—CVAD方案可改善LBL患者预后。

Objective To investigate the clinical characteristics and prognostic factors of patients with lymphoblastie lymphoma （LBL）. Methods Records of 43 Patients with LBL （39 T-LBL and 4 B-LBL） treated from Jan. 2003 to Dec. 2011 in Tumor Hospital, Xinjiang Medical University, were retrospectively analyzed. 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 used for survival analysis and Cox regression model for multivariate analysis. Results 30 patients were male and 13 female. The median age at diagnosis was 13 years （ranged from 5 years to 46）. 38 cases （88. 3%） were in stage Ⅲ or Ⅳ at diagnosis. 22 cases（51.2%） had mediastinal masses. The response rate （RR） for the whole group was 55.8%, complete remission （CR） rate 34.9%. With a median follow-up duration of 11 months （range 4--66 months）, The 1--year and 2--year overall survival （OS） rates were 55% and45% respectively. In univariate analysis, performance status （PS）, B symptoms, mediastinal masses, involved bone marrow, central nervous system （CNS） involvement, serum lactate dehydrogenase （LDH） levels, choice of therapy modality and achievement of remission were statistically significantly correlated to the prognosis. In univariate analysis, elevated LDH level, choice of therapy modality and achievement of remission were statistically significant prognostic factors. Conclusion LBL is more common in children and adolescents and has poor prognosis in Xinjiang region. Modified BFM 90 protocol and Hyper-CVAD regimes can improve the efficacy and survival of LBL.