Chen Yan, Sun Xiao-Fei, Zhen Zi-Jun, Wang Juan, Zhu Jia, Lu Su-Ying, Sun Fei-Fei, Zhang Fei, Li Peng-Fei, Cai Rui-Qing
State Key Laboratory of Oncology in South China, Department of Pediatric Oncology, Sun Yat-sen University Cancer Center, Guangzhou, Guangdong 510060, P. R. China.
Chin J Cancer. 2013 Oct;32(10):561-6. doi: 10.5732/cjc.012.10198. Epub 2013 Apr 1.
Pediatric diffuse large B-cell lymphoma (DLBCL) is a highly aggressive disease with unique clinical characteristics. This study analyzed the germinal-center type B-cell (GCB) classification and clinical characteristics of Chinese pediatric DLBCL. A total of 76 patients with DLBCL newly diagnosed in Sun Yat-sen University Cancer Center between February 2000 and May 2011, with an age younger than 18 years, were included in the analysis. The male/female ratio was 3.47:1. The median age was 12 years (range, 2 to 18 years), and 47 (61.8%) patients were at least 10 years old. Of the 76 patients, 48 (63.2%) had stage III/IV disease, 9 (11.8%) had bone marrow involvement, 1 (1.3%) had central nervous system (CNS) involvement, and 5 (6.6%) had bone involvement. The GCB classification was assessed in 45 patients: 26 (57.8%) were classified as GCB subtype, and 19 (42.2%) were classified as non-GCB subtype. The modified B-NHL-BFM-90/95 regimen was administered to 50 patients, and the 4-year event-free survival (EFS) rate was 85.8%. Among these 50 patients, 31 were assessed for the GCB classification: 17 (54.8%) were classified as GCB subtype, with a 4-year EFS rate of 88.2%; 14 (45.2%) were classified as non-GCB subtype, with a 4-year EFS rate of 92.9%. Our data indicate that bone marrow involvement and stage III/IV disease are common in Chinese pediatric DLBCL patients, whereas the percentage of patients with the GCB subtype is similar to that of patients with the non-GCB subtype. The modified B-NHL-BFM-90/95 protocol is an active and effective treatment protocol for Chinese pediatric patients with DLBCL.
儿童弥漫性大B细胞淋巴瘤（DLBCL）是一种具有独特临床特征的高度侵袭性疾病。本研究分析了中国儿童DLBCL的生发中心B细胞（GCB）分类及临床特征。纳入分析的患者为2000年2月至2011年5月间在中山大学肿瘤防治中心新诊断的76例年龄小于18岁的DLBCL患者。男女比例为3.47∶1。中位年龄为12岁（范围2至18岁），47例（61.8%）患者年龄至少10岁。76例患者中，48例（63.2%）为Ⅲ/Ⅳ期疾病，9例（11.8%）有骨髓受累，1例（1.3%）有中枢神经系统（CNS）受累，5例（6.6%）有骨受累。对45例患者进行了GCB分类评估：26例（57.8%）被分类为GCB亚型，19例（42.2%）被分类为非GCB亚型。50例患者接受了改良的B-NHL-BFM-90/95方案治疗，4年无事件生存率（EFS）为85.8%。在这50例患者中，31例进行了GCB分类评估：17例（54.8%）被分类为GCB亚型，4年EFS率为88.2%；14例（45.2%）被分类为非GCB亚型，4年EFS率为92.9%。我们的数据表明，骨髓受累和Ⅲ/Ⅳ期疾病在中国儿童DLBCL患者中很常见，而GCB亚型患者的比例与非GCB亚型患者相似。改良的B-NHL-BFM-90/95方案是治疗中国儿童DLBCL患者的一种积极有效的治疗方案。
