基于结内或结外原发部位的弥漫性大B细胞淋巴瘤的临床特征与预后：中国一家单中心1085例世界卫生组织分类病例分析
Clinical features and outcomes of diffuse large B-cell lymphoma based on nodal or extranodal primary sites of origin: Analysis of 1,085 WHO classified cases in a single institution in China.
作者信息
Shi Yuankai, Han Ying, Yang Jianliang, Liu Peng, He Xiaohui, Zhang Changgong, Zhou Shengyu, Zhou Liqiang, Qin Yan, Song Yongwen, Liu Yueping, Wang Shulian, Jin Jing, Gui Lin, Sun Yan
机构信息
Department of Medical Oncology, Beijing Key Laboratory of Clinical Study on Anticancer Molecular Targeted Drugs, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
Departement of Radiation Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
出版信息
Chin J Cancer Res. 2019 Feb;31(1):152-161. doi: 10.21147/j.issn.1000-9604.2019.01.10.
OBJECTIVE
To explore the clinicobiologic features and outcomes of diffuse large B-cell lymphoma (DLBCL) patients in China according to the primary site.
METHODS
A total of 1,085 patients diagnosed with DLBCL in National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College during a 6-year period were enrolled. Their clinical characteristics and outcomes were analyzed according to the primary site.
RESULTS
In the 1,085 patients, 679 (62.6%) cases were nodal DLBCL (N-DLBCL) and 406 cases (37.4%) were extranodal DLBCL (EN-DLBCL). The most common sites of N-DLBCL were lymphonodus (64.8%), Waldeyer's ring (19.7%), mediastinum (12.8%) and spleen (2.7%), while in EN-DLBCL, stomach (22.4%), intestine (16.0%), nose and sinuses (8.9%), testis (8.4%), skin (7.9%), thyroid (6.9%), central nervous system (CNS) (6.4%), breast (5.7%), bone (3.4%), and salivary gland (2.7%) were most common. N-DLBCL patients tend to present B symptoms, bulky disease, and elevated LDH more often, while age >60 years, extranodal sites >1, Ann Arbor stage I or II, bone marrow involvement, and Ki-67 index >90% were usually seen in EN-DLBCL. The 5-year overall survival (OS) rate and progression-free survival (PFS) rate for all patients were 62.5% and 54.2%. The 5-year OS rate for patients with N-DLBCL and EN-DLBCL were 65.5% and 56.9% (P=0.008), and the 5-year PFS were 57.0% and 49.0% (P=0.020). Waldeyer's ring originated DLBCL possessed the highest 5-year OS rate (83.6%) and PFS rate (76.9%) in N-DLBCL. The top five EN-DLBCL subtypes with favorable prognosis were stomach, breast, nose and sinuses, lung, salivary gland, with 5-year OS rate: 70.3%, 69.6%, 69.4%, 66.7% and 63.6%, respectively. While CNS, testis, oral cavity and kidney originated EN-DLBCL faced miserable prognosis, with 5-year OS rate of 26.9%, 38.2%, and 42.9%.
CONCLUSIONS
In our study, primary sites were associated with clinical characteristics and outcomes. Compared with EN-DLBCL, N-DLBCL had better prognosis.
目的
根据原发部位探讨中国弥漫性大B细胞淋巴瘤（DLBCL）患者的临床生物学特征及预后。
方法
纳入中国医学科学院肿瘤医院、北京协和医学院国家癌症中心在6年期间诊断为DLBCL的1085例患者。根据原发部位分析其临床特征及预后。
结果
1085例患者中，679例（62.6%）为结内DLBCL（N-DLBCL），406例（37.4%）为结外DLBCL（EN-DLBCL）。N-DLBCL最常见的部位是淋巴结（64.8%）、韦氏环（19.7%）、纵隔（12.8%）和脾脏（2.7%），而EN-DLBCL中，胃（22.4%）、肠（16.0%）、鼻及鼻窦（8.9%）、睾丸（8.4%）、皮肤（7.9%）、甲状腺（6.9%）、中枢神经系统（CNS）（6.4%）、乳腺（5.7%）、骨（3.4%）和唾液腺（2.7%）最常见。N-DLBCL患者更常出现B症状、大包块病变和乳酸脱氢酶升高，而年龄>60岁、结外部位>1个、Ann Arbor分期I或II期、骨髓受累及Ki-67指数>90%在EN-DLBCL中较为常见。所有患者的5年总生存率（OS）和无进展生存率（PFS）分别为62.5%和54.2%。N-DLBCL和EN-DLBCL患者的5年OS率分别为65.5%和56.9%（P=0.008），5年PFS分别为57.0%和49.0%（P=0.020）。韦氏环起源的DLBCL在N-DLBCL中5年OS率（83.6%）和PFS率（76.9%）最高。预后良好的前5种EN-DLBCL亚型是胃、乳腺、鼻及鼻窦、肺、唾液腺，5年OS率分别为：70.3%、69.6%、69.4%、66.7%和63.6%。而CNS、睾丸、口腔和肾脏起源的EN-DLBCL预后较差，5年OS率为26.9%、38.2%和42.9%。
结论
在我们的研究中，原发部位与临床特征及预后相关。与EN-DLBCL相比，N-DLBCL预后更好。
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