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  • Title: Immune deficiency in the X-linked lymphoproliferative syndrome. I. Epstein-Barr virus-specific defects.
    Author: Harada S, Sakamoto K, Seeley JK, Lindsten T, Bechtold T, Yetz J, Rogers G, Pearson G, Purtilo DT.
    Journal: J Immunol; 1982 Dec; 129(6):2532-5. PubMed ID: 6292290.
    Abstract:
    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.
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