Carbonnel F, Lavergne A, Messing B, Tsapis A, Berger R, Galian A, Nemeth J, Brouet J C, Rambaud J C
Service de Gastroentérologie, Hôpital Saint-Lazare, Paris, France.
Cancer. 1994 Feb 15;73(4):1286-91. doi: 10.1002/1097-0142(19940215)73:4<1286::aid-cncr2820730425>3.0.co;2-9.
Primary T-cell lymphoma of the small intestine is rare, and most cases have proved rapidly fatal.
We describe a case of lymphoma involving the small intestine uniformly and extensively in a 28-year-old man on initial examination seen with long-standing diarrhea, malabsorption, and recurrent episodes of intestinal obstruction. Clinical remission was obtained with pentostatin (2'-deoxycoformycin, supplied by Professor Catovsky, London UK) after the patient had failed to improve under conventional chemotherapy. Tumor specimens as well as mesenteric lymph node, liver, and bone marrow specimens were studied with conventional pathology and immunochemistry. Additionally, mesenteric lymph nodes and peripheral blood cells were studied for T-cell receptor (TCR) gene rearrangement and karyotype.
Lymphoma cells were small T-lymphocytes with irregular pleomorphic nuclei, bearing the CD3, CD4 and TCR alpha-beta phenotype. Peripheral-blood cytology and bone marrow biopsy were normal. Southern blot analysis of the TCR beta-chain gene revealed the same monoclonal rearrangement in the mesenteric lymph nodes and peripheral blood lymphocytes. An as yet undescribed t(4;16)(q26;p13) translocation, involving the region where the interleukin-2 (IL-2) gene has been mapped, was present in the mesenteric lymph nodes and peripheral blood lymphocytes.
We believe this is the first description of an extensive, small intestinal lymphoma of low-grade malignancy made up of monoclonal T-cells with a TCR alpha-beta and helper/inducer phenotype, associated with a novel chromosomal translocation.
原发性小肠T细胞淋巴瘤罕见，多数病例已证实进展迅速且致命。
我们描述了一例28岁男性患者，初诊时小肠广泛且均匀地受累于淋巴瘤，伴有长期腹泻、吸收不良及反复发作的肠梗阻。在患者接受传统化疗无效后，使用喷司他丁（2'-脱氧助间型霉素，由英国伦敦的Catovsky教授提供）获得了临床缓解。对肿瘤标本以及肠系膜淋巴结、肝脏和骨髓标本进行了常规病理学和免疫化学研究。此外，对肠系膜淋巴结和外周血细胞进行了T细胞受体（TCR）基因重排和核型分析。
淋巴瘤细胞为小T淋巴细胞，核呈不规则多形性，具有CD3、CD4和TCRα-β表型。外周血细胞学和骨髓活检均正常。TCRβ链基因的Southern印迹分析显示，肠系膜淋巴结和外周血淋巴细胞中存在相同的单克隆重排。肠系膜淋巴结和外周血淋巴细胞中存在一种尚未描述的t(4;16)(q26;p13)易位，涉及白细胞介素-2（IL-2）基因所在区域。
我们认为这是首次描述由具有TCRα-β和辅助/诱导表型的单克隆T细胞组成的广泛的低度恶性小肠淋巴瘤，并伴有一种新的染色体易位
