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Germinal-center type B-cell classification and clinical characteristics of Chinese pediatric diffuse large B-cell lymphoma:a report of 76 cases
Authors:Yan Chen  Xiao-Fei Sun  Zi-Jun Zhen  Juan Wang  Jia Zhu  Su-Ying Lu  Fei-Fei Sun  Fei Zhang  Peng-Fei Li  Rui-Qing Cai
Affiliation:Yan Chen (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China); Xiao-Fei Sun (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China); Zi-Jun Zhen (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China); Juan Wang (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China); Jia Zhu (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China); Su-Ying Lu (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China); Fei-Fei Sun (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China); Fei Zhang (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China); Peng-Fei Li (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China); Rui-Qing Cai (State Key Laboratory of 0ncology in South China, Guangzhou, Guangdong 510060, P. R. China);
Abstract: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.
Keywords:Non-Hodgkin's lymphoma   diffuse large B-cell lymphoma   pediatric   chemotherapy   germinal-center type B-cell classification   clinical characteristics
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