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Background: Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease associated with human T-lymphotropic virus type 1 infection, with a very high prevalence in tropical areas but exceptionally rare in Europe and Western countries.Case presentation: We describe a challenging case of ATLL in a young male patient with Brazilian origin and adopted as a child by an Italian family, presenting to our clinic with atypical T-lymphocytosis and life-threatening lung infections.Conclusions: Diagnosis of ATLL outside of endemic areas can be difficult, requiring a high index of clinical suspicion with careful evaluation of the patient’s clinical history. Prognosis is affected by disease stage at presentation and degree of immunosuppression. Few effective treatments are available, although new molecular insights have highlighted the role of host immune response and immune checkpoint blockade inhibitors, given the overexpression of PD-L1 on lymphoma cells and on microenvironment cells.

Chromatin modifiers are emerging as determinants of many types of cancer, including anaplastic large cell lymphoma (ALCL), a family of highly heterogeneous T-cell lymphomas for which therapeutic options are still limited. We recently identified the DNA helicase HELLS - a member of the SWI/SNF2 family- as a genetic vulnerability of ALK -ALCLs. HELLS is a multifunctional chromatin remodeler that affects genomic instability. Although its transcriptional function has been suggested, no clues on how HELLS controls transcription are currently available.