Reversible Neutrophil Defect in Ataxia Telangiectasia

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Abstract
Ataxia telangiectasia is a complex syndrome characterized by cerebellar ataxia, oculocutaneous telangiectasias, and frequent sinopulmonary infections. The pathogenesis of this condition remains unknown, but the increased susceptibility to infections has been related to IgA and IgE deficiency, as well as to faulty cellular immunity. According to the W.H.O. classification of primary immunodeficient disorders, both B-cells (Bursal) and T-cells (Thymic) are defective in this syndrome. Recently, Feigin, et al. described a case of ataxia telangiectasia associated with granulocytopenia. We would like to report a case of ataxia telangiectasia where a reversible, apparently intrinsic, neutrophil defect was detected along with some of the immune defects that usually accompany this syndrome.

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