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.
D000293:Adolescent; D001260:Ataxia Telangiectasia / Q000150:complications*; D002648:Child; D006801:Humans; D007945:Leukemia, Lymphoid / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008297:Male