单形性亲上皮性肠道T细胞淋巴瘤临床病理分析

摘要/Abstract

摘要：

目的探讨单形性亲上皮性肠道T细胞淋巴瘤（monomorphic epitheliotropic intestinal T-cell lymphoma，MEITL）的临床病理特点、免疫组化、分子遗传学改变、治疗及预后。方法选择betway必威登陆网址第二附属医院2018年1月—2022年6月的3例MEITL患者，应用HE染色、免疫组织化学（immunohistochemistry，IHC）染色、原位杂交、T细胞受体（T cell receptor，TCR）基因克隆性重排检测等方法观察并总结其组织病理学特点，回顾患者的诊疗经过及预后，分析患者的临床病理学特点。结果3例MEITL患者的病变部位均位于小肠，其中1例累及多段小肠、乙状结肠、子宫、膀胱；3例患者入院时均有腹胀、腹痛、肠穿孔及弥漫性腹膜炎，其中2例有发热。3例患者均行手术治疗。1例患者接受化疗，3例患者均已死亡。病理特征：肿瘤细胞弥漫性生长，浸润肠壁全层，肿瘤细胞形态较单一，小至中等大，细胞质淡染，核圆形、核仁不明显，染色质细腻；可见“亲上皮现象”，可见核分裂像，可见坏死形成。IHC检测结果显示：CD3、CD8、CD56、Bcl?2、TIA?1呈阳性表达；2例CD4阴性，1例部分阳性；2例CD20阴性，1例部分弱阳性；2例Granzyme B阴性，1例散在阳性；CD45RO部分阳性；CD21、PAX?5、CD5呈阴性表达，Ki?67增殖指数约40% ~ 70%。3例患者EBER原位杂交检测结果均显示阴性，无EB病毒感染。TCR重排检测均阳性。结论METCL是罕见的高侵袭性T细胞淋巴瘤，多位于小肠，易发生肠道穿孔，进展快、预后差。METCL诊断需结合临床表现、病理学特征和免疫组化、基因检测结果等进行综合判断。发生严重并发症者预后极差，早期诊断、及时手术、化疗反应好的患者生存期相对较长。

关键词:单形性亲上皮性肠道T细胞淋巴瘤,免疫组织化学,原位杂交,T细胞受体

Abstract:

ObjectiveTo summarize the clinical pathological characteristics，immunohistochemistry， molecular genetic changes， treatment and prognosis of monomorphic epitheliotropic intestinal T-cell lymphoma （MEITL）.MethodsA total of 3 patients with MEITL in the Second Affiliated Hospital of Shandong First Medical University were collected from 2018 to 2022． Hematoxylin eosin staining， immunohistochemistry staining， in situ hybridization and clonal rearrangement of T cell receptor （TCR） gene were used to detect their histopathological features. The diagnosis and treatment as well as prognosis of the patients were reviewed， and their clinical pathological characteristics were analyzed.ResultsAll three patients lesions were located in the small intestine，and one of them infiltrated multisegmental small intestine， sigmoid colon， uterus and bladder. They had abdominal distension， abdominal pain， intestinal perforation and diffuse peritonitis at the time of admission， and two of them had fever. They were all treated by surgery， one patient also received chemotherapy， and all three patients died. Pathological characteristics showed that tumor cells grew diffusely and infiltrated the whole layer of the intestinal wall. The tumor cells had a single morphology， small to medium sizes， lightly stained cytoplasm， round nuclei， inconspicuous nucleoli， and exquisite chromatin. “Epitheliotropic phenomenon” were seen， together with mitotic figures and the formation of necrosis. Immunohistochemistry showed that CD3， CD8， CD56， Bcl-2 and TIA-1 were positive； CD4 was negative in 2 cases and partially positive in 1 case．CD20 was negative in 2 cases and partially weak positive in 1 case；Granzyme B was negative in 2 cases and scatteredly positive in 1 case；CD45RO was partially positive in all 3 cases；CD21， PAX-5 and CD5 were negative， the proliferation index of Ki-67 was 40% ~ 70% . In situ hybridization showed that small RNA encoded by Epstein Barr virus was negative and there was no EB virus infection．TCR rearrangement was positive in all 3 cases.ConclusionMETCL is a rare highly aggressive T-cell lymphoma， most of which is located in the small intestine， prone to intestinal perforation， with rapid progression and poor prognosis. The diagnosis should be comprehensively judged based on the combination of clinical manifestations， pathological features， immunohistochemistry， and gene detection results. The prognosis of patients with serious complications is very poor. The patients who have an early diagnosis， timely operation and good chemotherapy reaction have a relatively long survival time.

Key words:monomorphic epitheliotropic intestinal T-cell lymphoma,immunohistochemistry,in situ hybridization,T cell receptor

刘蕾, 李小龙, 高英华, 郑家辉, 杨帆, 吴若晨, 张晓宇, 张树鹏. 单形性亲上皮性肠道T细胞淋巴瘤临床病理分析[J]. betway必威登陆网址（betway.com ）学报, 2022, 43(12): 932-937.

Lei LIU, Xiaolong LI, Yinghua GAO, Jiahui ZHENG, Fan YANG, Ruochen WU, Xiaoyu ZHANG, Shupeng ZHANG. Clinicopathological features of monomorphic epitheliophilic intestinal T-cell lymphoma[J]. Journal of Shandong First Medical Unversity & Shandong Academy of Medical Sciences, 2022, 43(12): 932-937.

图/表12

图1肿瘤细胞破坏小肠黏膜，肠黏膜上皮内见肿瘤性淋巴细胞浸润，可见“亲上皮现象”（HE， × 200）

图2肿瘤细胞小至中等，形态相对单一（HE， × 400）

图3肿瘤细胞可见星空现象（HE， × 400）

图4CD8 细胞膜阳性（IHC， × 400）

图5CD3 细胞膜阳性（IHC， × 400）

图6CD56 细胞膜阳性（IHC， × 400）

图7TIA-1 细胞质阳性（IHC， × 400）

图8Bcl-2细胞浆阳性（IHC， × 400）

图9Ki-67 细胞核阳性，增殖指数高（IHC， × 400）

图10CD20 阴性（IHC， × 400）

图11CD5 阴性（IHC， × 400）

表13例单形性亲上皮性肠道T细胞淋巴瘤患者TCR基因重排检测结果

病例	β链	γ链	δ链
病例1		γ链Vγlf、Vγ10-Jγ阳性	δ链Vδ-Jδ、Dδ-Dδ、Dδ-Jδ阳性
病例2	β链Dβ-Jβ阳性	γ链Vγlf、Vγ10-Jγ阳性	δ链Vδ-Jδ、Dδ-Dδ、Dδ-Jδ阳性
病例3	β链Dβ-Jβ、Vβ-Jβ阳性	γ链Vγlf、Vγ10-Jγ阳性

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