细胞毒性T细胞淋巴瘤134例临床病理学特点及预后  

Clinicopathological features and prognosis of cytotoxic T-cell lymphoma:analysis of 134 cases

作　　者：侯卫华 周小鸽[2] 谢建兰[2] 郑媛媛[2] 张燕林[2] 王续[3] Hou Weihua;Zhou Xiaoge;Xie Jianlan;Zheng Yuanyuan;Zhang Yanlin;Wang Xu(Department of Pathology,Pingdingshan Medical District(Former the 152 Central Hospital),the 989 Hospital of PLA Joint Logistic Support Force,Pingdingshan 467099,Henan,China;Department of Pathology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China;Department of Pathology,the Fourth Hospital of Hebei Medical University,Shijiazhuang 050011,China)

机构地区：[1]解放军联勤保障部队第九八九医院平顶山医疗区(原第一五二中心医院)病理科,平顶山467099 [2]首都医科大学附属北京友谊医院病理科,北京100050 [3]河北医科大学第四医院病理科,石家庄050011

出　　处：《中华病理学杂志》2022年第4期290-295,共6页Chinese Journal of Pathology

摘　　要：目的探讨细胞毒性T细胞淋巴瘤(cytotoxic T-cell lymphoma,CTL)临床病理学特点及预后。方法回顾性收集2008至2020年首都医科大学附属北京友谊医院、解放军联勤保障部队第九八九医院(原第一五二中心医院)和河北医科大学第四医院共计134例CTL患者的临床病理资料,检测肿瘤细胞的免疫表型、EB病毒感染状态和T细胞受体(TCR)克隆性,分析患者的临床病理特征和预后。结果134例CTL患者中,男女比例1.7∶1.0,中位年龄为49.5岁(范围3~83岁),60岁以下者100例(74.6%)。有B症状者占46.9%(53/113)。多数患者表现为全身浅表淋巴结肿大。按Ann Arbor系统分期,Ⅰ~Ⅱ期占36.8%(39/106),Ⅲ~Ⅳ期占63.2%(67/106)。伴有结外侵犯者占51.6%(66/128)。脾脏受累占24.2%(31/128)。形态学上表现为异型淋巴细胞弥漫性生长,浸润破坏正常组织结构。免疫组织化学染色显示肿瘤细胞阳性表达T细胞抗原(CD2、CD3、CD5、CD7),表达减弱或表达缺失其中一个或多个抗原者占72.0%(77/107)。根据肿瘤细胞CD4和CD8的表达量分组,其中CD8+>CD4+组70例(52.2%)。T细胞胞质内抗原1(TIA1)和颗粒酶B表达率分别为99.2%(119/120)和79.8%(95/119)。CD20异常表达率27.6%(37/134),CD56阴性。Ki-67阳性指数中位值为45.0%(范围5%~80%)。EB病毒编码的小RNA原位杂交检测均为阴性。TCR基因克隆性检测,所检的49例样本TCR基因重排结果均为阳性,其余病例未检测。91例患者获得随访,中位随访36个月(范围1~240个月),死亡40例(44.0%)。疗效达完全缓解者23例(含13例局限性单一结外肿块患者)。患者3年和5年总生存率分别为53.5%和49.4%。单因素分析结果显示B症状、脾脏受累、结外侵犯、临床分期、CD8+>CD4+表型、CD20异常表达和Ki-67阳性指数(>60%)对患者总生存率的影响差异具有统计学意义(P<0.05)。将单因素分析有统计学意义的指标纳入多因素Cox回归模型,结果显示脾脏受累和CD8+>CD4+表型是影�Objective To investigate the clinicopathological features and prognosis of cytotoxic T-cell lymphoma(CTL).Methods The clinicopathological data of 134 CTL patients in Beijing Friendship Hospital Affiliated to Capital Medical University,the 989 Hospital of PLA Joint Logistics Support force(formerly the 152 Hospital)and the Fourth Hospital of Hebei Medical University from 2008 to 2020 were retrospectively collected.Immunophenotype,Epstein-Barr virus infection status and T cell receptor(TCR)clonality of tumor cells were assessed,and clinicopathological features and prognosis of patients were analyzed.Results Among the 134 CTL patients,the male to female ratio was 1.7∶1.0,the median age was 49.5 years(range 3-83 years),and 100 cases(74.6%)were under 60 years old.Forty-six point nine percent of the patients(53/113)had B symptoms.Most of the patients presented with systemic superficial lymphadenopathy.According to the Ann Arbor staging system,36.8%(39/106)of the patients were in stageⅠ-Ⅱ,and 63.2%(67/106)in stageⅢ-Ⅳ.The rate of extranodal involvement was 51.6%(66/128).Spleen was involved in 24.2%(31/128)of the cases.Morphology showed diffuse growth of abnormal lymphocytes,infiltrating and destroying normal tissue structure.Immunohistochemical staining showed that tumor cells expressed T cell antigens(CD2,CD3,CD5,and CD7),and 72.0%(77/107)of them had decreased or lost expression of one or more antigens.According to the numbers of CD4 and CD8 expression in tumor cells,70 cases(52.2%)were grouped into CD8+>CD4+group.The expression rates of TIA-1 and granzyme B were 99.2%(119/120)and 79.8%(95/119),respectively.CD20 abnormal expression rate was 27.6%(37/134)and CD56 was negative in all cases.The median Ki-67 proliferative index was 45.0%(range 5%-80%).In situ hybridization of small RNA encoded by Epstein-Barr virus was negative.Clonal TCR gene rearrangement analysis was performed on 49 cases and was positive in all cases.Ninety-one patients were followed up for a median of 36 months(range,1 to 240 months),and 40 of

关 键 词：淋巴瘤 T细胞 外周 淋巴瘤 T细胞 T淋巴细胞 细胞毒性 预后 

分 类 号：R733.1[医药卫生—肿瘤]