Shibayama Haruna, Imadome Ken-Ichi, Onozawa Erika, Tsuzura Akiho, Miura Osamu, Koyama Takatoshi, Arai Ayako
Department of Hematology, Tokyo Medical and Dental University.
Department of Laboratory Molecular Genetics of Hematology, Tokyo Medical and Dental University.
Rinsho Ketsueki. 2017;58(6):583-588. doi: 10.11406/rinketsu.58.583.
Chronic active Epstein-Barr virus infection (CAEBV) is a disease characterized by clonally proliferating and activated EBV-infected T or NK cells accompanied by chronic inflammation and T- or NK-cell neoplasms. However, the mechanism for developing CAEBV has not been clarified to date. Because the decreased number or inactivation of EBV-specific cytotoxic T lymphocytes (CTLs) resulted in the development of EBV-positive B-cell neoplasms, we investigated the number of CTLs in CAEBV patients using the tetrameric complexes of HLA-restricted EBV-specific peptides. Among the seven patients examined, EBV-specific CTLs were detected in the peripheral blood mononuclear cells (PBMCs) of four cases but were not detected in three cases. The ratio of EBV-specific CTLs in PBMCs tended to be higher in the patients with active disease than in those with inactive disease. In two patients in whom EBV-specific CTLs had not been detected, CTLs appeared after the eradication of EBV-infected T cells by allogeneic bone marrow transplantation. These results suggested that the failure of CTLs had a role in developing CAEBV, although the induction number and function of EBV-specific CTLs might vary in each patient.
慢性活动性EB病毒感染（CAEBV）是一种以克隆性增殖和活化的EB病毒感染的T或NK细胞为特征，伴有慢性炎症和T或NK细胞肿瘤的疾病。然而，迄今为止，CAEBV的发病机制尚未阐明。由于EB病毒特异性细胞毒性T淋巴细胞（CTL）数量减少或失活导致EB病毒阳性B细胞肿瘤的发生，我们使用HLA限制性EB病毒特异性肽的四聚体复合物研究了CAEBV患者的CTL数量。在所检查的7例患者中，4例患者的外周血单个核细胞（PBMC）中检测到EB病毒特异性CTL，3例未检测到。PBMC中EB病毒特异性CTL的比例在活动性疾病患者中往往高于非活动性疾病患者。在2例未检测到EB病毒特异性CTL的患者中，通过异基因骨髓移植清除EB病毒感染的T细胞后出现了CTL。这些结果表明，CTL功能缺陷在CAEBV的发生中起作用，尽管每个患者中EB病毒特异性CTL的诱导数量和功能可能有所不同。
