Ataxia‐telangiectasia and acute lymphoblastic leukemia

Abstract

Lymphoreticular malignancies occur frequently in association with ataxia‐telangiectasia (A‐T). This paper presents one new case and reviews all 19 known cases of A‐T and acute lymphoblastic leukemia (ALL). The leukemia is associated with poor prognostic factors: male sex, older age, high white blood cell count, and T or B‐cells. There have been no long‐term survivors; recently, prolonged remissions have been attained. These patients appear to be at special risk for infections secondary to the immunodeficient state associated with A‐T and ALL therapy. A heightened sensitivity to the neurotoxic effects of vinca alkaloids and central nervous system irradiation seems to be present. Copyright © 1980 American Cancer Society