Tsutsumi Y, Inada K, Morita K, Suzuki T
Department of Pathology, Tokai University School of Medicine, Kanagawa, Japan.
Jpn J Clin Oncol. 1996 Aug;26(4):264-72. doi: 10.1093/oxfordjournals.jjco.a023226.
Two adult cases of extranodal malignant lymphoma diffusely involving the intestinal wall are reported. Lymphoblastic lymphoma in case 1 (68-year-old male) and small lymphocytic lymphoma in case 2 (48-year-old male) were initially manifested as protein-losing enteropathy or malabsorption syndrome. In case 1, the patient died of massive ascites and intestinal bleeding 10 months after presentation. Autopsy revealed diffuse and extensive lymphomatous involvement of the small and large intestine, peritoneum and liver. The patient in case 2, showing low-grade diffuse lymphomatous invasion throughout the small bowel mucosa without involving other organs, is currently being followed up. Surface marker studies revealed previously undescribed phenotypes, such as CD4/CD8 double-positive blastoid T cells in case 1, and CD8-positive suppressor/killer T cells in case 2. T-cell receptor alpha/beta was expressed on the lymphoma cells in both cases. Epstein-Barr virus infection was not evident. The gut-associated nature of the T-lymphoma cells, possibly of extrathymic origin, is discussed.
报告了2例成人结外恶性淋巴瘤弥漫性累及肠壁的病例。病例1（68岁男性）为淋巴母细胞淋巴瘤，病例2（48岁男性）为小淋巴细胞淋巴瘤，最初均表现为蛋白丢失性肠病或吸收不良综合征。病例1中，患者在就诊10个月后死于大量腹水和肠道出血。尸检显示小肠、大肠、腹膜和肝脏均有弥漫性广泛的淋巴瘤浸润。病例2中的患者表现为整个小肠黏膜的低度弥漫性淋巴瘤浸润，未累及其他器官，目前正在随访中。表面标志物研究揭示了以前未描述的表型，如病例1中的CD4/CD8双阳性母细胞样T细胞，以及病例2中的CD8阳性抑制/杀伤T细胞。两例病例的淋巴瘤细胞均表达T细胞受体α/β。未发现明显的爱泼斯坦-巴尔病毒感染。讨论了T淋巴瘤细胞可能起源于胸腺外的肠道相关性。
