Abstract
ATAXIA telangiectasia is an inherited disorder characterized by progressive cerebellar ataxia and oculocutaneous telangiectasia.<sup>1-3</sup>Generally these patients have recurrent sinopulmonary infections, and defects in cellular immunity and in the immunoglobulin system have been recently documented.<sup>4-9</sup>An unusual propensity for the development of neoplasia has become apparent.<sup>2-4,10-14</sup>In previous cases the neoplasms have been discovered close to or at the time of death, obviating therapy. Since this is apparently the first instance in which a patient with ataxia telangiectasia has been treated for a lymphosarcoma, we wish to call attention to the severe untoward effect of a partial therapeutic dose of x-irradiation. In addition, we found typical lesions of tuberous sclerosis in the brain and unusual hypertrophic cells in the renal tubules. These findings, associated with the development of a lymphosarcoma, suggest a generalized disturbance in the metabolic control of many cell types. <h3>Report of a Case</h3> A
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
