Ataxia-telangiectasia and acute lymphoblastic leukemia

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.