ATAXIA-TELANGIECTASIA

Ataxia-telangiectasia is a complex syndrome that includes a very high cancer risk in children with a progressive cerebellar ataxia, the onset of which occurs in early infancy. Ocular telangiectasiae often do not appear until several years after the ataxia. The most common type of malignancy is lymphoma, usually of the B-cell type. Leukemias also occur. Failure to diagnose ataxia-telangiectasia in an infant with lymphoma or leukemia may result in radiation therapy with conventional dosages, which is contraindicated in ataxia-telangiectasia patients.