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Two patients with the clinical diagnosis of ataxia-telangectasia are reported. Both had a mixed partial immunodeficiency characterized by selective absence or deficiency of IgA and anergy to cutaneous antigens. During the course of their disease both patients developed a poorly differentiated lymphocytic lymphoma of intraabdominal location. One of the patients died from infectious complications after receiving the first course of chemotherapy, while the second one remains alive under treatment. The high incidence of lymphoreticular neoplasms in early life in these patients with a genetically deficient immune system is commented upon along with the fact that, in spite of that, the pathogenesis of the syndrome remains unknown.
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