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56例伴有纵隔肿块的淋巴瘤患者的临床特征及预后

[Clinical Characteristics and Prognosis of 56 Lymphoma Patients with Mediastinal Masses].

作者信息

Yu Hui, Wang Li, Zhu Hua-Yuan, Wu Wei, Liang Jin-Hua, Yin Hua, Fan Lei, Li Jian-Yong, Xu Wei

机构信息

Department of Hematology, The First Affiliated Hospital of Nanjing Medical University (Jiangsu Province People Hospital), Nanjing 210029, Jiangsu Province, China.

Department of Hematology, The First Affiliated Hospital of Nanjing Medical University (Jiangsu Province People Hospital), Nanjing 210029, Jiangsu Province, China,E-mail:

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2020 Apr;28(2):495-499. doi: 10.19746/j.cnki.issn.1009-2137.2020.02.023.

DOI:10.19746/j.cnki.issn.1009-2137.2020.02.023
PMID:32319385
Abstract

OBJECTIVE

To analyze the incidence, clinical characteristics, treatment efficacy and prognosis of lymphoma patients with mediastinal masses.

METHODS

The clinical data of 56 lymphoma patients with mediastinal masses treated in our hospital from February 2007 to January 2018 were collected, and the clinical characteristics, typing, staging, treatment efficacy as well as prognosis analyzed retrospectively.

RESULTS

Among the 56 patients, male and female were 25 (45%) cases and 31(55%) cases, respectively. The median age was 31 (17-72) years old, out of them 48 (86%) patients aged below 40 years old. According to pathological classification, 9 (16.1%) cases were diffuse large B cell lymphoma, not otherwise specified (DLBCL-NOS), 26(46.4%) cases were primary mediastinal large B cell lymphoma (PMBCL), 17 (30.4%) cases were classical Hodgkin's lymphoma (cHL), and 4 (7.1%) cases were primary mediastinal grey zone lymphoma (MGZL). All the patients were treated with chemotherapy or immuno-chemotherapy.The median follow-up time was 54.5(11-149) months. In cHL group, 10 cases received involved field radiotherapy (IFRT), 10 (58.8%) cases achieved complete remission (CR), 2 (11.8%) cases achieved partial remisson (PR), and 5 (29.4%) patients experienced progressive disease (PD) , 6 patients with relapsed/refractory disease received autologous hematopoietic stem cell transplantation (auto-HSCT), the 1, 2, and 5 year overall survival (OS) rates of cHL group were 94.1%, 88.2%, and 67.2%, respectively. In MGZL group, 3 cases combined with IFRT, 1 case with auto-HSCT, 2 (50%) cases achieved CR, 2 (50%) cases experienced PD, the 1, 2, 5 year OS rates of MGZL group were 66.7%, 66.7%, and 33.3%, respectively. In PMBCL group, 8 cases combined with IFRT, 7 cases with auto-HSCT, 22 (84.0%) cases achieved CR, 2 (8.0%) cases achieved PR, 1 (4.0%) case was stable disease (SD) and 1 (4.0%) case experienced PD, the 1, 2 year OS rates of PMBCL group were both 100%, and 5 year OS rate was 95.7%. In DLBCL-NOS group, 3 cases combined with IFRT, 2 cases with auto-HSCT, 4 (44.5%) cases achieved CR, 2 (22.2%) cases achieved PR, 1 (11.1%) case was stable disease (SD) and 2 (22.2%) cases experienced PD, the 1 year OS rate of DLBCL-NOS cohort was 100%, 2, and 5 year OS rates were both 77.8%. There was a significant difference of OS rate among these 4 group (P<0.05). In intra-group comparison, OS rate in PMBCL group were significantly longer than both MGZL group (P<0.01) and cHL group(P<0.05), and showed no significant difference from DLBCL-NOS group (P>0.05).

CONCLUSION

Mediastinal masses are characterized by unique clinical features due to their unique anatomical location. Several B cell originated lymphomas including cHL,MGZL,PMBCL and DLBCL-NOS, manifested primary or secondary mediastina involvement, tend to occur in young adults and have similar clinical features. However, their therapeutic response was significantly different. The prognosis of PMBCL is prior to MGZL and cHL.

摘要

目的

分析纵隔肿块型淋巴瘤患者的发病率、临床特征、治疗疗效及预后。

方法

收集2007年2月至2018年1月在我院治疗的56例纵隔肿块型淋巴瘤患者的临床资料,回顾性分析其临床特征、分型、分期、治疗疗效及预后。

结果

56例患者中,男性25例(45%),女性31例(55%)。中位年龄为31(17 - 72)岁,其中48例(86%)患者年龄在40岁以下。根据病理分类,9例(16.1%)为弥漫性大B细胞淋巴瘤,非特指型(DLBCL - NOS),26例(46.4%)为原发性纵隔大B细胞淋巴瘤(PMBCL),17例(30.4%)为经典型霍奇金淋巴瘤(cHL),4例(7.1%)为原发性纵隔灰色地带淋巴瘤(MGZL)。所有患者均接受化疗或免疫化疗。中位随访时间为54.5(11 - 149)个月。在cHL组中,10例接受累及野放疗(IFRT),10例(58.8%)达到完全缓解(CR),2例(11.8%)达到部分缓解(PR),5例(29.4%)患者疾病进展(PD),6例复发/难治性疾病患者接受自体造血干细胞移植(auto - HSCT),cHL组1、2、5年总生存率(OS)分别为94.1%、88.2%、67.2%。在MGZL组中,3例联合IFRT,1例接受auto - HSCT,2例(50%)达到CR,2例(50%)疾病进展(PD),MGZL组1、2、5年OS率分别为66.7%、66.7%、33.3%。在PMBCL组中,8例联合IFRT,7例接受auto - HSCT,22例(84.0%)达到CR,2例(8.0%)达到PR,1例(4.0%)病情稳定(SD),1例(4.0%)疾病进展(PD),PMBCL组1、2年OS率均为100%,5年OS率为95.7%。在DLBCL - NOS组中,3例联合IFRT,2例接受auto - HSCT,4例(44.5%)达到CR,2例(22.2%)达到PR,1例(11.1%)病情稳定(SD),2例(22.2%)疾病进展(PD),DLBCL - NOS队列1年OS率为100%,2、5年OS率均为77.8%。这4组的OS率差异有统计学意义(P<0.05)。组内比较,PMBCL组的OS率显著长于MGZL组(P<0.01)和cHL组(P<0.05),与DLBCL - NOS组无显著差异(P>0.05)。

结论

纵隔肿块因其独特的解剖位置具有独特的临床特征。几种B细胞起源的淋巴瘤,包括cHL、MGZL、PMBCL和DLBCL - NOS,表现为原发性或继发性纵隔受累,倾向于发生在年轻人中且具有相似的临床特征。然而,它们的治疗反应有显著差异。PMBCL的预后优于MGZL和cHL。

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