first author

Tatton-Brown-Rahman syndrome (TBRS) is an uncommon overgrowth and neurodevelopmental condition arising from heterozygous mutations in DNMT3A, which confer a predisposition to hematologic cancers. We report a 10-year-old boy diagnosed with classical Hodgkin lymphoma who was subsequently identified to harbour the pathogenic DNMT3A p.Arg882Cys variant. He responded completely to standard ABVD chemotherapy. This observation broadens the known tumour spectrum of TBRS to include Hodgkin lymphoma and underscores the oncogenic impact of DNMT3A disruption. Lifelong surveillance, coordinated multidisciplinary follow-up, and genetic counselling are crucial for early identification of potential malignant transformation in individuals with TBRS.