Preservation of natural killer and interleukin-2 activated killer cell activity in ataxia-telangiectasia with T cell deficiency

Abstract

Peripheral blood mononuclear cells (PBMs) from 6 patients with ataxia-telangiectasia (AT) were studied by 5 kinds of cell-mediated cytotoxicity systems. Decrease in cell mediated lympholysis (CML) activity to allogeneic lymphocytes was observed in all 6 AT patients who had low numbers of OKT-3+ cells. These patients also showed decreased proliferative responses to phytohemagglutinin stimulation and allogeneic lymphocytes. In contrast, antibody-dependent cell-mediated cytotoxicity (ADCC) activity and natural killer (NK) activity were comparable with those in normal controls. In addition, PBMs from these AT patients activated by in vitro stimulation with allogeneic PBMs or interleukin-2 were able to acquire lytic activity against NK-insensitive target cells. The phenotypes of these effectors determined by complement-mediated lysis were OKT-3- and Leu-11+, suggesting that they were derived from NK cell lineage. Thus, AT patients with severe T cell defects were found to maintain a normal range of NK, ADCC, MLC-activated and lymphokine-activated killer activity.