Abnormal anti-Epstein Barr virus antibodies in carriers of the X-linked lymphoproliferative syndrome and in females at risk.
Abnormal anti-Epstein Barr virus antibodies in carriers of the X-linked lymphoproliferative syndrome and in females at risk.
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X连锁淋巴增殖综合征携带者和高危女性体内抗EB病毒抗体异常。
DOI：
10.4049/jimmunol.128.2.904
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发表时间：
1982
影响因子：
4.4
通讯作者：
D. Purtilo
中科院分区：
文献类型：
--
作者：
K. Sakamoto;J. Seeley;T. Lindsten;J. Sexton;J. Yetz;M. Ballow;D. Purtilo
X连锁淋巴增殖综合征（XLP）的无症状半合子女性携带者对EBV有异常的抗体反应。这表明该缺陷的部分表达导致其受影响的儿子患上由 EB 病毒引起的危及生命的疾病。在 1 至 5 年间，对 12 名女性专性携带者及其 7 名女儿（处于危险中的女性）的 65 份血清样本进行了 EBV 特异性抗体检测。异常定性抗病毒衣壳抗原 (VCA) IgG 滴度比正常对照高出近四倍，两名携带者具有持久性 IgM 抗 VCA 抗体，三分之二具有持久性 IgA 抗 VCA 抗体，一半的女性具有早期抗原 (EA) 滴度。七名女性中有五名表现出类似的持续模式。相比之下，即使在高度暴露于病毒的情况下，未受影响的家庭成员和 23 名正常对照均未表达 VCA 的 IgA 或 IgM 滴度，并且仅在一名对照中检测到抗 EA。因此，这些发现可能有助于检测该综合征的携带者。据报道，患者和其他免疫抑制个体中存在与携带者模式相似的异常抗 EBV 滴度，这表明存在活动性病毒感染。
The asymptomatic hemizygous female carriers of the X-linked lymphoproliferative syndrome (XLP) have abnormal antibody responses to EBV. This suggests partial expression of the defect that leads to EBV-provoked life-threatening diseases in their affected sons. EBV specific antibodies were measured in 65 serum samples of 12 obligate carrier females and seven of their daughters (females at risk) during periods ranging from 1 to 5 yr. Abnormal qualitative antiviral capsid antigen (VCA) IgG titers were nearly fourfold higher than normal controls, two carriers had persistent IgM anti-VCA antibody, two-thirds had persistent IgA anti-VCA antibody, and half of the women had titers to early antigen (EA). Five of seven females exhibited a similar persistent pattern. In contrast, none of the unaffected family members nor 23 normal controls expressed IgA or IgM titers to VCA even with high exposure to the virus, and anti-EA was detected in only one control. Therefore, these findings may prove useful for detecting carriers of the syndrome. Abnormal anti-EBV titers similar to the carrier pattern have been reported in patients and other immunosuppressed individuals, and are indicative of active viral infection.