• Marco Calvaruso, Alessandro Gulino, Salvatore Buffa, Carla Guarnotta, Giovanni Franco, Matilde Cacciatore, Maria Grazia Bonura, Vito Franco, and Ada Maria Florena.
• Laboratorio di Tecnologie Oncologiche - HSR Giglio, C. da Pietrapollastra-Pisciotto , Cefalù , Italy.
• Leuk. Lymphoma. 2014 Nov 1; 55 (11): 2457-65.

AbstractPeripheral T-cell lymphomas (PTCLs) are a heterogeneous group of lymphoid neoplasms characterized by aggressive clinical behavior and dismal prognosis. Hepatosplenic γδ T-cell lymphoma (γδ-HSTL) is a particular form of PTCL that arises from a small subset of γ/δ T-cell receptor-expressing lymphocytes. γδ-HSTL has a rapidly progressive course and poor outcome due also to its refractoriness to conventional chemotherapy regimens. The very low incidence of γδ-HSTL, along with its propensity to mimic different pathological entities, makes this lymphoma a true diagnostic challenge. In this review, we highlight the biological and clinical features of γδ-HSTL that contribute to making this lymphoma a mostly incurable disease. Moreover, we provide a new insight into the crosstalk between HSTL clones and the bone marrow, liver and spleen vascular microenvironment, in which neoplastic cells reside and proliferate. We further discuss γδ-HSTL associated molecules that might be proposed as potential targets for novel therapeutic approaches.

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