女性生殖系统弥漫大B细胞淋巴瘤患者临床病理特征及预后分析  

Clinicopathological characteristics and prognostic analysis of patients with female genital tract diffuse large B-cell lymphoma

作　　者：石子旸 王玥 施晴 王硕 张慕晨 沈容 赫洋[1] 裘惠玲 易红梅 董磊[1] 王黎[1] 程澍[1] 许彭鹏[1] 赵维莅[1] Shi Ziyang;Wang Yue;Shi Qing;Wang Shuo;Zhang Muchen;Shen Rong;He Yang;Qiu Huiling;Yi Hongmei;Dong Lei;Wang Li;Cheng Shu;Xu Pengpeng;Zhao Weili(Department of Hematology,State Key Laboratory of Medical Genomics,Shanghai Institute of Hematology,Rui Jin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China)

机构地区：[1]上海交通大学医学院附属瑞金医院血液科,医学基因组学国家重点实验室,上海血液学研究所,上海200025

出　　处：《白血病．淋巴瘤》2022年第9期527-532,共6页Journal of Leukemia & Lymphoma

基　　金：国家自然科学基金(82130004、81830007、81670176、82070204)。

摘　　要：目的探讨女性生殖系统弥漫大B细胞淋巴瘤(DLBCL)患者的临床病理特征、基因突变谱及预后相关因素。方法回顾性分析2003年10月至2021年10月上海交通大学医学院附属瑞金医院收治的30例女性生殖系统DLBCL患者的临床病理资料。采用靶向测序检测55个淋巴瘤相关基因,评估患者的基因突变情况。采用Kaplan-Meier法进行生存分析,采用Cox比例风险模型分析预后影响因素。结果30例女性生殖系统DLBCL患者的中位发病年龄为58岁(23~77岁)。8例(32%)首发症状为腹痛、腹胀及腹部包块。13例(45%)病变累及附件区(含卵巢、输卵管),其中9例为单侧受累。21例(70%)存在多结外受累,22例(73%)Ann Arbor分期Ⅲ~Ⅳ期,8例(27%)美国东部肿瘤协作组(ECOG)评分≥2分,22例(73%)乳酸脱氢酶(LDH)升高,21例(70%)国际预后指数(IPI)评分3~5分。30例患者中,11例(37%)采取手术治疗;所有患者均接受R-CHOP方案为基础的化疗。30例均可评估疗效,完全缓解率为83%(25/30),5年无进展生存(PFS)率为69.7%,5年总生存(OS)率为79.6%。单因素分析显示,ECOG评分≥2分与较差的OS相关(P=0.048)。30例患者中,原发7例(23%),继发23例(77%)。继发患者中Ann Arbor分期Ⅲ~Ⅳ期、IPI评分3~5分、LDH升高者比例均高于原发患者(均P<0.001),但二者PFS和OS差异均无统计学意义(P值分别为0.261、0.671)。16例患者的靶向测序结果显示,PIM1、MYD88、KMT2D、TP53、CARD11、CCND3和GNA13突变率均>20%,其中TP53突变与患者较差的PFS和OS相关(P值分别为0.012、0.002)。结论女性生殖系统DLBCL是一种罕见的侵袭性结外DLBCL,原发和继发患者生存预后相似,PIM1、MYD88、TP53基因高频突变可能为治疗提供新的方向。Objective To investigate the clinicopathological characteristics,gene mutation profile,and prognostic factors of diffuse large B-cell lymphoma(DLBCL)in female genital tract.Methods A retrospective analysis was performed on the clinicopathological data of 30 patients with female genital tract DLBCL who were admitted to Rui Jin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from October 2003 to October 2021.Targeted sequencing was used to detect 55 lymphoma-related genes,and the gene mutation status of patients was evaluated.Kaplan-Meier method was used for survival analysis,and prognostic factors were analyzed by Cox proportional hazards model.Results The median age of 30 female genital tract DLBCL patients at diagnosis was 58 years old(23-77 years old).The initial symptoms mainly included abdominal pain,distension,and masses(8 cases,32%).Tumors most commonly located in the adnexal region(including ovaries and fallopian tubes)(13 cases,45%),of which 9 cases were unilateral.Twenty-one cases(70%)had multiple extra-nodal involvements,22 cases(73%)had Ann Arbor stageⅢ-Ⅳ,8 cases(27%)had Eastern Cooperative Oncology Group(ECOG)score of≥2,and 22 cases(73%)had elevated lactate dehydrogenase(LDH),21 cases(70%)had International Prognostic Index(IPI)score of 3-5.Within 30 patients,11 patients(37%)received surgery,and all patients received R-CHOP regimen-based chemotherapy.All 30 cases were evaluated for efficacy,the complete remission rate was 83%(25/30),the 5-year progression-free survival(PFS)rate was 69.7%,and the 5-year overall survival(OS)rate was 79.6%.Univariate analysis showed that ECOG score≥2 was associated with worse OS(P=0.048).Among the 30 patients,7 patients(23%)were primary and 23 patients(77%)were secondary.The proportions of patients with Ann Arbor stageⅢ-Ⅳ,IPI score 3-5 and elevated LDH in secondary patients were higher than those in primary patients(all P<0.001),but there were no significant differences in PFS and OS between the two(P values were 0.261 and 0.671).The ta

关 键 词：淋巴瘤 大B细胞 弥漫性 女性生殖系统 临床病理学特征 基因突变谱 预后 

分 类 号：R737.3[医药卫生—肿瘤]