单形性嗜上皮肠道T细胞淋巴瘤的临床特征分析  

Clinicopathological features of monomorphic epitheliophilic intestinal T-cell lymphoma

作　　者：张炼 喻芳 韦菊英[1] 谢万灼[1] 叶琇锦[1] 许改香[1] 俞文娟[1] 佟红艳[1] ZHANG Lian;YU Fang;WEI Juying;XIE Wanzhuo;YE Xiujin;XU Gaixiang;YU Wenjuan;TONG Hongyan(Department of Hematology,the First Affiliated Hospital of Zhejiang University School of Medicine,Hangzhou 310003,China;不详)

机构地区：[1]浙江大学医学院附属第一医院血液科,杭州310003 [2]衢州市中医医院血液肿瘤科 [3]浙江大学医学院附属第一医院病理科

出　　处：《浙江医学》2023年第5期487-492,503,共7页Zhejiang Medical Journal

基　　金：国家中医临床研究基地业务建设科研专项(JDZX2015115)。

摘　　要：目的 探讨单形性嗜上皮性肠道T细胞淋巴瘤(MEITL)的临床特点及分期、病理学表现,以期提高临床医生对该病的认识。方法 回顾性分析2020年1月至2022年1月浙江大学医学院附属第一医院血液科收治的7例MEITL患者。收集整理患者基本人口学资料、临床表现、病理学结果、EBER原位杂交及TCR基因重排等分子生物学检测。分析MEITL的临床、病理特征。结果 7例患者中男2例,女5例;年龄55~67岁,中位年龄64岁;病程1~6个月,中位数2个月;腹痛6例,1例腹泻合并低钾血症起病,无乳糜泻。发病部位小肠4例,十二指肠、空肠及结肠各1例。乳酸脱氢酶升高3例,轻度贫血2例。癌胚抗原均未见升高。糖类抗原125、糖类抗原19-9及血清铁蛋白异常升高2例。7例骨髓学检查均无侵犯。3例在手术前行PET-CT检查,见受累处18F-氟脱氧葡萄糖(FDG)明显摄取增高,SUVmax波动在15.20~18.46。Lugano分期Ⅱ-1期1例、Ⅲ期1例、Ⅳ期5例。T细胞淋巴瘤预后指标(PIT)2分1例、3分6例。2例为内镜活检、5例行外科手术病理证实。6例行外科肠肿物切除手术,其中3例因继发小肠肠穿孔或梗阻行急诊手术,3例加结肠病变部分切除,肿瘤直径5~10 cm 1例,>10 cm 2例。光镜下瘤细胞呈单一性、大小为小至中等。免疫表型:CD3阳性(7/7)、CD8阳性(7/7)、CD56阳性(6/7)、TIA-1阳性(5/7);CD5阴性(6/6)、CD30阴性(5/5);1例CD20反常阳性表达。Ki-67指数中位数为70%,7例EBER阴性,2例TCR基因重排阳性。行基因检测1例:KRAS、FAT1、KMT2A、KMT2B、SETD2基因高频突变。7例均行化疗,包括为CHOP、AZA+CHOP、ECHOP、GVD联合西达苯胺等方案;1例行自体干细胞移植。7例均得到随访(末次随访时间2022年1月),死亡4例,存活3例。结论 MEITL罕见,高度恶性,进展迅速,无特异的临床表现,病死率高,基因重排等分子生物学检测是重要手段。Objective To analyze the clinicopathological features of monomorphic epitheliophilic intestinal T-cell lymphoma(MEITL).Methods Seven patients with MEITL admitted in Department of Hematology,the First Affiliated Hospital of Zhejiang University School of Medicine from January 2020 to January 2022 were included in the study.Demographic data,clinical manifestations,histopathological findings,and results of EBER in situ hybridization,T cell receptor(TCR)gene rearrangement and other molecular biological indicators of patients were analyzed.Results There were 2 males and 5 females with a median age of 64 years(55-67 years).The duration of disease ranged from 1 to 6 months with a median of 2 months.Six cases complained abdominal pain and 1 case started with diarrhea and hypokalemia,without celiac disease.The lesions were located in small intestine for 4 cases,while in duodenum,jejunum or colon for 1 case,respectively.Elevated lactate dehydrogenase was observed in 3 cases,and mild anemia in 2.Serum CEA level was not significantly elevated;CA-125,CA19-9 or ferritin were abnormally elevated in 2 cases,respectively.Bone marrow was not involved in all patients as shown in pathological examination.Three patients underwent PET-CT scanning,which showed intense uptake of FDG in continuous lesions,and the SUVmax score ranged 15.2-18.46.There were 1 case staged II,1 case staged III and 5 cases staged IV according to the criteria of Lugano staging.PIT score was 2 in 1 case and 3 in 6 cases.Two cases confirmed by endoscopic biopsy,and 5 cases by surgical pathology.The bowel tumor was resected in 6 patients,3 of whom underwent emergency surgery due to secondary intestinal perforation or obstruction;the other 3 underwent partial resection of colon lesions,the diameter of the tumor was 5-10 cm in 1 case and larger than 10 cm in 2 cases.Consistent small to moderately large tumor cell infiltration was observed under light microscopy.The immune markers were:CD3^(+)(7/7),CD8+(7/7),CD56+(6/7),TIA-1+(5/7);CD5-(6/6),CD30-(5/5);1 case had abnor

关 键 词：单形性嗜上皮肠道T细胞淋巴瘤 T细胞抗原受体 分子生物学特征 诊断 

分 类 号：R735[医药卫生—肿瘤]