T-Cell Receptors in Ataxia–Telangiectasia

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Abstract
With reference to the article by Carbonari and colleagues (Jan. 11 issue) describing a relative increase in γ/δ T cells in patients with ataxia—telangiectasia,1 recent work by me and my collaborators has demonstrated a similar relative increase in these cells in the peripheral blood of patients with the Chédiak—Higashi syndrome. This is an immunodeficiency disorder involving impairment of cytotoxic activity, which is characterized by large, abnormal lysosomal granules in all granule-containing cells.2 The mean (±SD) ratio of γ/δ-bearing cells to α/β-bearing cells in patients with ataxia—telangiectasia (n = 10)1 is 0.230±0.190 (range, 0.075 to 0.600), and the ratio in patients with the Chédiak—Higashi syndrome (n = 5) is 0.243±0.122 (range, 0.105 to 0.406).