骨骼肌细胞毒性T细胞淋巴瘤临床病理学特点  

Clinicopathological features and prognosis of the skeletal-muscle cytotoxic T-cell lymphoma

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,989 Hospital of PLA Joint Logistics Support Force(Formerly 152 Hospital),Pingdingshan 467099,Henan Province,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,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》2021年第11期1246-1251,共6页Chinese Journal of Pathology

基　　金：首都卫生发展科研专项(2018-1-2151)。

摘　　要：目的探讨骨骼肌细胞毒性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—2019年首都医科大学附属北京友谊医院和解放军联勤保障部队第九八九医院(原第一五二中心医院)14例骨骼肌CTL和47例非骨骼肌结外CTL患者的临床资料,镜下观察肿瘤形态,检测肿瘤细胞的免疫表型、EB病毒(EBV)感染状态和T细胞受体(TCR)克隆性,比较分析两组患者的临床病理特征和预后。结果骨骼肌CTL占本组结外CTL的23.0%(14/61)。患者中位年龄45.5岁(11~76岁),男性6例,女性8例。临床上主要表现为局部生长的无痛性肿块。有B症状者占比为2/14。肿瘤分别发生于颊部(7例)、舌部(4例)、下唇(3例)及左上臂(2例),其中有2例均累及两个部位。临床分期ⅠE期10例,ⅡE期4例。与非骨骼肌结外CTL比较,骨骼肌CTL多无B症状、临床分期低、主要累及面颊及口腔(舌部和唇部,P<0.05)。形态学上表现为骨骼肌组织内异型淋巴细胞弥漫性浸润。免疫组织化学检测,肿瘤阳性表达T细胞抗原(CD2、CD3、CD5、CD7),其中一个或多个抗原表达减弱或表达缺失者占比为11/14。所有病例均表达T细胞胞质内抗原-1、颗粒酶B和CD8(CD8^(+)>CD4^(+)模式),CD56阴性。Ki-67阳性指数中位数为35.0%(5%~60%)。所有病例EBER原位杂交检测阴性。除6例未检测外,其余病例TCR基因重排克隆性分析检测结果为阳性。10例获得随访,中位随访时间为40个月(10~67个月)。10例患者完全缓解,5年生存率为100%,与非骨骼肌结外CTL(5年总生存率35.9%)比较,差异有统计学意义(χ^(2)=8.277,P=0.004)。结论骨骼肌CTL多发生于面颊和口腔,肿瘤细胞具有侵嗜骨骼肌或肌炎样的形态学特征,具有CD8^(+)>CD4^(+)和细胞毒性分子模式的免疫表型,临床分期低,预后较好。Objective To investigate the clinicopathological features and prognosis of skeletal-muscle cytotoxic T-cell lymphoma(CTL).Methods The clinical data of 14 cases of skeletal muscle CTL and 47 cases of non-skeletal muscle extranodal CTL patients in Beijing Friendship Hospital Affiliated to Capital Medical University and the 989 Hospital of the joint logistics support force of the people′s Liberation Army(the former 152 hospital)from 2008 to 2019 were collected retrospectively.Immunophenotype,EBV infection status and T-cell receptor(TCR)clonality of tumor cells were evaluated.The clinicopathological features and prognosis of the two groups were compared.Results Skeletal-muscle CTL accounted for 23.0%(14/61)of extranodal CTL in the same period.The median age of the patients was 42.3 years(range 11-76 years),including six males and eight females.The main clinical manifestations were painless masses.Two patients(2/14)had B symptoms.The tumors occurred in the cheek(7 cases),the tongue(4 cases),the lower lip(3 cases)and the left upper arm(2 cases),and in two cases had two sites.Ten cases were of stageⅠE and four cases stageⅡE.Compared with non-skeletal-muscle extranodal CTL,many patients of skeletal-muscle CTL did not have B symptoms,the clinical stage was lower,and the tumor mainly involved the oral cavity(cheek,tongue and lip,P<0.05).Morphologically,the tumor showed diffuse infiltration of heterotypic lymphocytes in skeletal muscle.Immunohistochemistry showed that in 11/14 cases,there were reduced or loss of expression of some the T cell antigens(CD2,CD3,CD5,CD7).TIA-1,Gr B and CD8(CD8^(+)>CD4^(+))were expressed in all cases,and CD56 was negative.The median Ki-67 proliferation index was 35.0%(range 5%-60%).EBER in situ hybridization was negative in all cases.The results of TCR clonality analysis showed clonal TCR gene rearrangement were detected in eight cases.The median follow-up time was 40 months(range 10-67 months).Ten patients were tumor free;the 5-year survival rate of skeletal-muscle CTL was 100%.Compared w

关 键 词：淋巴瘤 T细胞 外周 淋巴瘤 肌 骨骼 预后 

分 类 号：R738[医药卫生—肿瘤]