Lindsten T, Seeley J K, Ballow M, Sakamoto K, St Onge S, Yetz J, Aman P, Purtilo D T
J Immunol. 1982 Dec;129(6):2536-40.
Surface phenotypic markers and the function of lymphocytes in patients affected with the X-linked lymphoproliferative syndrome (XLP) were studied. This syndrome is characterized by a defective response to infection with Epstein Barr virus (EBV). Normal numbers of B and T cells were detected with anti-Ig and monoclonal OKT3 antisera, respectively. T cell subset values, however, were persistently altered: cells reacting with OKT8 were significantly elevated in five of nine patients, accompanied by a slight decrease in the percentage of OKT4-positive cells, leading to abnormally low OKT4 to OKT8 ratios. One patient had a high OKT4 to OKT8 ratio due to low number of OKT8-positive cells. Lymphocytes from patients showed normal proliferation after stimulation with T and B cell mitogens. In contrast, Ig synthesis by lymphocytes after stimulation with B cell mitogens was markedly deficient: low or undetectable levels of one or all classes of Ig were detected, whereas cell lines established from EBV-infected B lymphocytes from patients produced normal quantities of Ig. These studies imply immune regulatory impairments in the patient with XLP.
对患有X连锁淋巴增殖综合征（XLP）患者的淋巴细胞表面表型标志物及功能进行了研究。该综合征的特征是对爱泼斯坦-巴尔病毒（EBV）感染反应缺陷。分别用抗Ig和单克隆OKT3抗血清检测到正常数量的B细胞和T细胞。然而，T细胞亚群值持续改变：9名患者中有5名与OKT8反应的细胞显著升高，同时OKT4阳性细胞百分比略有下降，导致OKT4与OKT8比值异常低。1名患者由于OKT8阳性细胞数量低而具有高OKT4与OKT8比值。患者的淋巴细胞在用T细胞和B细胞有丝分裂原刺激后显示正常增殖。相反，淋巴细胞在用B细胞有丝分裂原刺激后的Ig合成明显不足：检测到一种或所有类别的Ig水平低或无法检测到，而从患者的EBV感染B淋巴细胞建立的细胞系产生正常量的Ig。这些研究表明XLP患者存在免疫调节障碍。
