单形性嗜上皮性肠道T细胞淋巴瘤4例及文献复习

Monomorphic epitheliotropic intestinal T-cell lymphoma:4 cases report and literature review

目的:结合既往病例报道和复习文献探讨单形性嗜上皮性肠道T细胞淋巴瘤(monomorphic epitheliotropic intestinal T-cell lymphoma,MEITL)的临床病理特点、临床诊疗特点及预后。方法:回顾分析4例MEITL的临床诊疗过程、组织形态学特征、免疫组化表型、分子病理结果、治疗过程及其预后,并结合既往病例报道和复习文献进行总结和分析。结果:本文报道的4例均为中老年患者,3例为男性,1例为女性,4例患者肿瘤均在小肠,病例3同时伴有乙状结肠肿瘤,临床表现主要为腹痛、肠梗阻、肠穿孔及盆腹腔积液;组织形态学特征为中等-大的异型淋巴细胞弥漫浸润肠壁全层组织,可见明显的肠隐窝及表面上皮内肿瘤亲上皮性生长。免疫组化检测METIL瘤细胞均表达CD3、CD43、TIA1、粒酶B、Bcl-2,原位杂交EBER均阴性。4例T淋巴细胞基因重排均阳性。结论:MEITL的发病率低,常发生在中老年男性的小肠,无特异性临床症状,需结合临床表现、组织学形态学特征、免疫组化表型及分子病理结果才能做出最终诊断,MEITL的预后极差且进展快,尚无有效的治疗方案,需提高对该疾病的认识和诊断水平。

Objective:To investigate the clinicopathological characteristics,clinical diagnosis and treatment,and prognosis of monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL) based on previous case reports and literature review.Methods:The clinical diagnosis and treatment process,histomorphological characteristics,immunohistochemical phenotype,molecular pathological results,treatment process and prognosis of 4 cases of MEITL were retrospectively analyzed,and the previous case reports and review literature were combined to summarize and analyze.Results:The 4 cases reported in this paper were all middle-aged and elderly patients,3 cases were male,1 case was female,4 cases had tumors in the small intestine,and case 3 was accompanied by sigmoid colon tumors.The main clinical manifestations were abdominal pain,intestinal obstruction,intestinal perforation,and pelvic and peritoneal effusion.Histomorphologically,medium to large heterotypic lymphocytes diffusely infiltrated the whole layer of intestinal wall,and obvious intestinal crypts and epithelial growth of tumors in the surface epithelium were observed.The expression of CD3,CD43,TIA1,granzyme B,and Bcl-2 in METIL tumor cells was detected by immunohistochemistry,and EBER in situ hybridization was negative.T lymphocyte gene rearrangement was positive in 4 cases.Conclusion:The incidence rate of MEITL is low.It often occurs in the small intestine of middle-aged and elderly men,and has no specific clinical symptoms.The final diagnosis can be made only by combining the clinical manifestations,histological and morphological characteristics,immunohistochemical phenotype and molecular pathological results.The prognosis of MEITL is extremely poor and progresses rapidly.There is no effective treatment plan for MEITL.It is necessary to improve the understanding and diagnosis of the disease.