Okano M, Osato T, Koizumi S, Imai S, Aya T, Fujiwara S, Mizuno F, Sakiyama Y, Matsumoto S, Sugawara O
AIDS Res. 1986 Dec;2 Suppl 1:S115-9.
Lymphomas occurred in 3 of 16 Japanese patients with ataxia telangiectasia (AT) and Wiskott-Aldrich syndrome (WAS). The patients had a persistently reactivated Epstein-Barr virus (EBV) infection with a remarkable decrease in virus-specific cellular immunity. In these patients, the B lymphocytes were more sensitive to EBV-induced events and to cellular proto-oncogene activation than seen in the healthy counterparts. This immunologic and genetic background was considered to explain the massive lymphoproliferation in these primary immunodeficiency disorders.
在16例患有共济失调毛细血管扩张症（AT）和威斯科特-奥尔德里奇综合征（WAS）的日本患者中，有3例发生了淋巴瘤。这些患者存在持续激活的爱泼斯坦-巴尔病毒（EBV）感染，且病毒特异性细胞免疫显著下降。在这些患者中，B淋巴细胞对EBV诱导的事件和细胞原癌基因激活比健康对照者更为敏感。这种免疫和遗传背景被认为可以解释这些原发性免疫缺陷疾病中的大量淋巴细胞增殖现象。
