摘要
背景:肠病型T细胞淋巴瘤(ETCL)是一种临床少见、预后差的疾病,了解其临床病理特征对诊断和治疗有重要的临床意义。目的:通过分析ETCL患者的临床表现、病理改变和分子生物学特征及其与EB病毒(EBV)感染的关系,提高临床医师和病理医师对ETCL的认识,以期改善患者的生存率。方法:回顾我院2001年7月～2004年1收治的5例ETCL患者的内镜活检或手术切除标本的临床和组织病理学表现;采用免疫组化方法标记CD45RO、CD3、CD56、CD20、CD79α、CD68、Lys;采用聚合酶链反应(PCR)检测EBV感染和T细胞受体(TCR)-γ基因重排情况。结果:5例ETCL患者均无肠病史,多发生于青年男性,4例发病年龄为24～38岁,病程进展迅猛,确诊后4个月内死亡。病变多发生于结肠和小肠,1例与胃部同时发生,均为多发性溃疡性病变。肿瘤细胞为多形性T细胞,组织学形态为多形性淋巴细胞弥散分布,有血管中心性浸润,淋巴上皮病变,大片或多灶性坏死为特征。5例患者肿瘤组织CD45RO和TCR-γ基因重排均为阳性,1例CD56阳性且EBV阳性。结论:ETCL多发生于青年男性,临床症状多无特异性,病程发展迅猛,预后差,病损肠管表现为多发性溃疡,TCR-γ链克隆性重排,多数为自然杀伤(NK)样T细胞淋巴瘤和外周T细胞淋巴瘤非特殊型;有EBV感染的ETCL为NK样T细胞淋巴瘤。
Background： Enteropathic type T-cell lymphoma （ETCL） is a rare disease with poor prognosis. Familiarizing its clinieo-pathologic characteristics is useful in its diagnosis and treatment. Aims： To analyze the clinical manifestations, pathological findings and molecular biological characteristics of patients with ETCL and its relationship with EB virus infection , and to promote its early recognition by the clinicians and pathologists, would improve the patients＇survival rate. Methods： To the biopsied or resected specimens of 5 cases of ETCL, the expressions of CD45RO, CD3, CD56, CD20, CD79α, CD68 and Lys were determined by immunohistochemical method, the Epstein-Barr virus （EBV） infection and T cell receptor （TCR）-γ gene rearrangement were detected by polymerase chain reaction （PCR）. Results： All five patients had no history of bowel diseases, it mainly occurred in young males, the age of onset in 4 cases were 24-38 years, and the disease progressed rapidly, 4 of them died 4 months after the definite diagnosis. The main pathological changs were ulceration lesions located in the colon and small intestine, one of them coexisted with multiple gastric ulcerations, ETCL consistsed of pleomorphic T lymphocytes with central vascular infiltration, lymphoepithelial lesion and massive or multifocal necrosis, Immunohistochemical studies showed positive CD45RO and TCR-γgene rearrangement in all cases, and one of them was additionally positive for both EBV and CD56. Conclusions： ETCL occurs most frequently in young males with nonspecific symptoms, but progresses rapidly resulting in poor prognosis. The intestinal lesions are characterized by multiple ulcerations with TCR-γ gene rearrangement, most of them are NK cell-like T-cell lymphomas and nonspecific peripheral T-cell lymphomas, the EBV infection can be detected in the former.
出处
《胃肠病学》
2005年第4期212-216,共5页
Chinese Journal of Gastroenterology
