Brouland J P, Molimard J, Nemeth J, Valleur P, Galian A
Service Central d'Anatomie et de Cytologie Pathologiques, Hôpital Lariboisière, Paris, France.
Virchows Arch A Pathol Anat Histopathol. 1993;423(6):513-7. doi: 10.1007/BF01606544.
A 34-year-old woman was hospitalized for the investigation of a one-month history of intestinal disorders, gastric heaviness and transitory icteric episodes. Extensive clinical investigations suggested the diagnosis of gall bladder carcinoma or sclerosing cholangitis. At laparotomy, the proximal part of common bile duct was markedly thickened by a white, firm, fish-flesh like tumour extending in to the cystic duct, gall bladder wall and to the liver. Histological study showed a diffuse lymphoid proliferation of the common bile duct mainly composed of small cells mixed with scattered large atypical cells. Immunohistochemistry revealed that most of the small cells expressed T-cell markers with predominant CD 4 and alpha-beta T-cell receptors and without phenotypic gap, whereas large atypical cells showed monotypic B phenotype with co-expression of mu and delta heavy chains and light lambda chain restriction. No evidence of primary nodal lymphoma was found during extensive clinical, radiological, sonographic or scanographic examinations. Sequential chemotherapy (MACOP-B) was instituted and the patient was still alive 4 years after diagnosis. Morphological and immunohistochemistry findings fulfilled criteria for a primary high grade B-cell lymphoma (centroblastic type, Kiel classification) from common bile duct concealed by numerous small reactive T-cells, so called T-cell rich B-cell lymphoma, not previously described in this location.
一名34岁女性因肠道紊乱、胃部沉重感及短暂黄疸发作1个月的病史而住院。广泛的临床检查提示胆囊癌或硬化性胆管炎的诊断。剖腹手术时，胆总管近端明显增厚，有一个白色、坚实、鱼肉样肿瘤，延伸至胆囊管、胆囊壁及肝脏。组织学研究显示胆总管弥漫性淋巴细胞增殖，主要由小细胞组成，混有散在的大的非典型细胞。免疫组化显示大多数小细胞表达T细胞标志物，以CD4和α-β T细胞受体为主，且无表型差异，而大的非典型细胞显示单克隆B表型，伴有μ和δ重链共表达及轻链λ链受限。在广泛的临床、放射学、超声或扫描检查中未发现原发性淋巴结淋巴瘤的证据。开始进行序贯化疗（MACOP-B），诊断后4年患者仍存活。形态学和免疫组化结果符合原发性高级别B细胞淋巴瘤（中心母细胞型， Kiel分类）的标准，该淋巴瘤位于胆总管，被大量小的反应性T细胞掩盖，即所谓的富含T细胞的B细胞淋巴瘤，此前该部位未见报道。
