Cellular mediated immune responses in vitro of six males with X-linked lymphoproliferative syndrome (XLP) were investigated. Impaired natural killing (NK) activity compared to seven controls (P < 0.001) and interferon-α activation (P < 0.011) were detected. However, normal numbers of lymphocytes expressing NK-associated antigens (Leu-7 and MO1) and large granular lymphocytes (LGL) were observed in all patients. Normal lymphocyte-mediated antibody-dependent cellular cytotoxicity (ADCC) (P < 0.531) was found. NK and ADCC activities have been regarded as being mediated by the same subsets of lymphocytes, however, results of this study do not support this hypothesis: lymphocytes with impaired NK but normal ADCC functions ( NK- K+) were found in males with XLP.
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