Mortality and Cancer Incidence in 263 Patients With Ataxia-Telangiectasia2

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Abstract
Mortality and cancer incidence were measured retrospectively in 263 ataxia-telangiectasia (A-T) homozygotes. For white and black A-T patients, respectively, all-cause mortality was 50 and 147 times higher than expected based on U.S. mortality rates. There were 52 primary cancers, representing a 61-fold cancer excess for white probands and a 184-fold excess for black probands. The cancer excess was most pronounced for lymphoma, with 252- and 750-fold excesses observed for whites and blacks, respectively. All the age-specific mortality and cancer incidence rates for blacks exceeded those for whites, and overall mortality was 3.0 times higher for black probands than for whites (P<.001), whereas cancer incidence was 2.2 times higher (P<.06). Among the white A-T patients, 36% of those who had died had lived at least until 20 years of age, and 33% of those still living were at least 20 years old.

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