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Ataxia telangiectasia (A-T) is a rare childhood autosomal recessive neurodegenerative chromosomal instability disorder. It is characterized by high risk of hematological malignancies with T-cell phenotype being the most common, which can present first before the diagnosis of A-T made. The chromosomal instability in A-T increases the toxicity to radio- chemotherapeutic agents, creating the treatment modification challenges and the deviation from the optimal management protocols. In this casedoi:10.22541/au.160610415.55719975/v1 fatcat:pwyb5px64vflbjjw3vzpwicpvi