Harada S, Sakamoto K, Seeley J K, Lindsten T, Bechtold T, Yetz J, Rogers G, Pearson G, Purtilo D T
J Immunol. 1982 Dec;129(6):2532-5.
Eleven males with XLP were evaluated for EBV-specific antibodies during periods of 2 to 7 yr. Variable responses to EBV-specific antigens were found. All 11 patients had subnormal anti-EBNA titers, which probably reflected a T cell deficiency. The patients showed four different patterns in their anti-VCA response: 1) two boys who had experienced malignant lymphoma mounted no antibodies at all; 2) two patients showed intermittent anti-VCA titers; 3) four males had persistently elevated anti-VCA titers; and 4) three patients showed normal anti-VCA titers. ADCC against EBV-infected cells was abnormally low in six patients and was elevated in two patients given gamma-globulin. ADCC titers did not correlate with anti-VCA titers. However, most patients with XLP failed to effect regression of autologous EBV-infected lymphoblastoid cell lines, indicating a deficiency in long-lived T cell-mediated immunity to EBV.
对11名X连锁淋巴增生性疾病（XLP）男性患者进行了为期2至7年的EB病毒特异性抗体评估。发现他们对EB病毒特异性抗原的反应各不相同。所有11名患者的抗EB核抗原（EBNA）滴度均低于正常水平，这可能反映了T细胞缺陷。患者的抗衣壳抗原（VCA）反应呈现出四种不同模式：1）两名曾患恶性淋巴瘤的男孩根本没有产生抗体；2）两名患者的抗VCA滴度呈间歇性；3）四名男性的抗VCA滴度持续升高；4）三名患者的抗VCA滴度正常。6名患者针对EB病毒感染细胞的抗体依赖细胞介导的细胞毒性（ADCC）异常低，而在两名接受γ球蛋白治疗的患者中ADCC升高。ADCC滴度与抗VCA滴度无关。然而，大多数XLP患者未能使自体EB病毒感染的淋巴母细胞系消退，这表明对EB病毒的长期T细胞介导免疫存在缺陷。
