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.
A prospective, randomized multicenter study was performed to evaluate the relative efficacy of two different concepts for early intensive therapy in a randomized trial of children with B-precursor acute lymphoblastic leukemia (ALL) at high risk (
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