Su I J, Lin K H, Chen C J, Tien H F, Hsieh H C, Lin D T, Chen J Y
Department of Pathology, National Taiwan University Hospital, Taipei, Republic of China.
Cancer. 1990 Dec 15;66(12):2557-62. doi: 10.1002/1097-0142(19901215)66:12<2557::aid-cncr2820661218>3.0.co;2-2.
Two childhood cases are reported of peripheral T-cell lymphoma; the neoplastic cells expressed activated CD8 (T8) phenotype and contained Epstein-Barr viral (EBV) DNA. Both patients had an aggressive and rapid clinical course despite chemotherapy. Elevated titers of antibodies to EBV-viral capsid antigen (greater than 640) and early antigen (greater than 10) were found in both patients. Histology revealed pleomorphic immunoblastic lymphoma with extensive necrosis in one case and an angioimmunoblastic lymphadenopathy-like pattern containing Reed-Sternberg-like giant cells in the other. Southern blot hybridization studies showed clonal rearrangement of the T-cell-receptor beta gene in both cases, and a cytogenetic study on one case revealed clonal structure abnormality involving chromosomes 1, 6, 7, 10, and 19. Analysis of the tumor DNA showed a high copy number of EBV genome per cell compared with that of Raji and Marmoset B 95.8 lines; the study for human T-cell leukemia virus type I was negative. The EBV genome was found by in situ hybridization in the tumor nuclei in both cases. In addition to Burkitt's lymphoma, T-cell lymphoma of the helper phenotype, and Hodgkin's disease, EBV can contribute to the development of CD8-positive aggressive T-cell lymphoma.
报告了2例儿童外周T细胞淋巴瘤病例；肿瘤细胞表达活化的CD8（T8）表型并含有EB病毒（EBV）DNA。尽管进行了化疗，但两名患者的临床病程均呈侵袭性且进展迅速。两名患者均发现EB病毒衣壳抗原抗体（大于640）和早期抗原抗体（大于10）滴度升高。组织学检查显示，1例为多形性免疫母细胞淋巴瘤伴广泛坏死，另1例为血管免疫母细胞性淋巴结病样模式，含有里德-施特恩贝格样巨细胞。Southern印迹杂交研究显示，两例均有T细胞受体β基因的克隆重排，1例的细胞遗传学研究显示涉及染色体1、6、7、10和19的克隆结构异常。肿瘤DNA分析显示，与Raji和狨猴B 95.8细胞系相比，每个细胞的EBV基因组拷贝数较高；人T细胞白血病病毒I型研究为阴性。两例均通过原位杂交在肿瘤细胞核中发现EBV基因组。除伯基特淋巴瘤、辅助表型T细胞淋巴瘤和霍奇金病外，EBV可促使CD8阳性侵袭性T细胞淋巴瘤的发生。
