灰区淋巴瘤的临床和病理特征以及预后分析  

Clinical and pathological characteristics and prognosis analysis of gray zone lymphoma

作　　者：刘霜竹 宗香萍 蔡文治[1] 何海菊 马朝霞 李嘉琦 李彩霞[1] 吴德沛[1] Liu Shuangzhu;Zong Xiangping;Cai Wenzhi;He Haiju;Ma Zhaoxia;Li Jiaqi;Li Caixia;Wu Depei(the First Affiliated Hospital of Soochow University,National Clinical Research Center for Hematologic Diseases,Jiangsu Institute of Hematology,Key Laboratory of Thrombosis and Hemostasis of Ministry of Health,State Key Laboratory of Radiation Medicine and Protection,Soochow University,Suzhou 215006,China)

机构地区：[1]苏州大学附属第一医院,国家血液系统疾病临床医学研究中心,江苏省血液研究所,国家卫生健康委员会血栓与止血重点实验室,放射医学与辐射防护国家重点实验室(苏州大学),苏州215006

出　　处：《中华医学杂志》2024年第1期69-73,共5页National Medical Journal of China

基　　金：国家重点研发计划(2022YFC2502700);国家自然科学基金(82020108003);江苏省血液病医学创新中心(CXZX202201);苏州市科技计划(SLT201911)

摘　　要：探索灰区淋巴瘤(GZL)的临床和病理特征以及治疗方法及预后。回顾性收集2016年12月至2022年12月就诊于苏州大学附属第一医院的10例GZL患者的临床资料,分析其临床及病理学特征、治疗方法及预后转归情况。随访截止时间为2022年12月31日,随访时间[M(Q_(1),Q_(3))]为40.0(28.3,59.8)个月。疗效评估分为完全缓解(CR)、部分缓解(PR)、疾病稳定(SD)、疾病进展(PD)。10例患者中男6例,女4例,年龄[M(Q_(1),Q_(3))]为33.5(27.3~39.5)岁。其中8例患者纵隔(胸腺)受累,7例伴结外受累。Ann Arbor分期局限期1例,进展期9例。4例患者免疫表型为CD20强表达,CD30表达,CD15不表达;6例患者免疫表型CD20表达强弱不等,CD30和CD15强表达。1例患者给予经典型霍奇金淋巴瘤(cHL)样免疫化疗后仅获得PR,后给予增强的弥漫大B细胞淋巴瘤(DLBCL)样免疫化疗,获得CR。5例患者给予增强的DLBCL样免疫化疗,获得CR。4例未获得CR的患者接受二线或三线挽救治疗后均获得CR。全部患者均给予自体造血干细胞移植(ASCT)巩固治疗,其中1例患者在随访33个月时复发死亡,其余患者目前均维持持续缓解状态。本研究发现GZL多发生于年轻患者,纵隔受累常见,诊断依赖病理形态和免疫表型。GZL可能对DLBCL样强化免疫方案更敏感,序贯ASCT巩固治疗可降低患者的复发风险。To explore the clinical and pathological characteristics as well as therapies and prognosis of gray zone lymphoma(GZL).The clinical data of 10 GZL patients admitted to the First Affiliated Hospital of Soochow University from December 2016 to December 2022 were retrospectively collected.The clinical and pathological characteristics,therapies and prognosis were analyzed.The cut-off time for follow-up visits was December 31,2022,and the median time for follow-up visits[M(Q_(1),Q_(3))]was 40.0(28.3,59.8)months.Treatment efficacy was divided into complete remission(CR),partial remission(PR),stable disease(SD)and progressive disease(PD).There were 6 males and 4 females,with a median age[M(Q_(1),Q_(3))]of 33.5(27.3-39.5)years.Among them,8 patients had mediastinal(thymus)involvement and 7 patients were accompanied with extranodal involvement.According to Ann Arbor staging,1 case was in the limited stage and 9 cases were in the progressive stage.The immunophenotypes of 4 patients were strong expression of CD20,expression of CD30,and no expression of CD15.The immunophenotypes of 6 patients were unequal expression of CD20 and strong expression of CD30 and CD15.One patient received classical hodgkin lymphoma(cHL)-like immunochemotherapy and only achieved PR,and then received enhanced diffuse large b-cell lymphoma(DLBCL)-like immunochemotherapy to achieve CR.Five patients received enhanced DLBCL-like immunochemotherapy for induction therapy and achieved CR.All 4 patients who did not achieve CR achieved CR after receiving second-line or third-line salvage therapy.All patients were given autologous stem cell transplantation(ASCT)for consolidation therapy.One patient relapsed and died during the follow-up visit in the 33rd month,and the remaining patients currently maintained a state of sustained remission.It is found that GZL mostly occurs in young patients,mediastinal involvement is common,and diagnosis relies on pathological morphology and immunophenotype.GZL may be more sensitive to DLBCL-like intensive immune regimens.Seque

关 键 词：灰区淋巴瘤 纵隔起病 联合免疫治疗 自体造血干细胞移植 

分 类 号：R733[医药卫生—肿瘤]