Prevalence and outcomes of cancer and treatment-associated toxicities for patients with ataxia telangiectasia.

ABSTRACT:

Background

Ataxia telangiectasia (A-T) is a DNA repair disorder with cancer predisposition.

Objective

We sought to characterize the prevalence and outcomes of hematologic and solid cancers and treatment-associated toxicities in individuals with A-T.

Methods

Data were retrospectively analyzed from the Johns Hopkins Ataxia Telangiectasia Clinical Center cohort. Cumulative incidence and standardized incidence ratios of cancer, survival probability after cancer diagnosis, and standardized mortality ratios were calculated. Cox regression estimated risk of death on the basis of chemotherapy (standard vs reduced) dosing, and multivariable logistic regression evaluated cancer risk associations with ataxia telangiectasia mutated (ATM) exons and variants.

Results

Eighty-

SUBMITTER: Magnarelli A 

PROVIDER: S-EPMC11915532 | biostudies-literature | 2025 Feb

REPOSITORIES: biostudies-literature