Shiratsuchi Motoaki, Suehiro Youko, Yoshikawa Yasuji, Ohshima Koichi, Shiokawa Satoshi, Nishimura Junji
Division of Clinical Immunology, Department of Immunobiology and Neuroscience, Medical Institute of Bioregulation, Kyushu University, Beppu, Japan.
Int J Hematol. 2004 Jan;79(1):44-7. doi: 10.1007/BF02983532.
Enteropathy-type T-cell lymphoma (ETCL) is a rare extranodal lymphoma that tends to disseminate into the intestines and other extranodal organs. We present a case of ETCL with involvement of the lungs and kidneys and report CC chemokine receptor 7 (CCR7) expression of lymphoma cells. A 73-year-old man was admitted to the hospital with a complaint of abdominal pain. Multiple ulcers and perforations were observed in the small intestine, and partial resection of the ileum was performed. Histological examination of the resected specimen revealed diffuse proliferation of atypical large lymphoid cells. The diagnosis was ETCL with dissemination into the lungs and kidney. Lymphoma cells of the small intestine and in pleural effusion were CD3+, CD4+, CD7+, CD8-, CD25-, CD56-, CD103 +/-, and TIA-1+. Rearrangement of the T-cell receptor beta gene was detected, and human T-lymphotropic virus was not integrated. Combination chemotherapy did not result in a sustained response. The results for CCR7 expression of lymphoma cells in the lung and pleural effusion were negative. Therefore we concluded that lymphoma cells did not migrate into the lymph nodes but instead spread into the extranodal organs.
肠病型T细胞淋巴瘤（ETCL）是一种罕见的结外淋巴瘤，易于扩散至肠道和其他结外器官。我们报告一例累及肺和肾的ETCL病例，并报道淋巴瘤细胞的CC趋化因子受体7（CCR7）表达情况。一名73岁男性因腹痛入院。小肠发现多处溃疡和穿孔，行回肠部分切除术。切除标本的组织学检查显示非典型大淋巴细胞弥漫性增殖。诊断为播散至肺和肾的ETCL。小肠及胸腔积液中的淋巴瘤细胞CD3+、CD4+、CD7+、CD8-、CD25-、CD56-、CD103+/-、TIA-1+。检测到T细胞受体β基因重排，未检测到人嗜T淋巴细胞病毒整合。联合化疗未产生持续缓解。肺及胸腔积液中淋巴瘤细胞的CCR7表达结果为阴性。因此我们得出结论，淋巴瘤细胞未迁移至淋巴结，而是扩散至结外器官。
