title: Challenges and new prospects in hepatosplenic γδ T-cell lymphoma.
abstract: Peripheral 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.
authors: Laboratorio di Tecnologie Oncologiche - HSR Giglio, C. da Pietrapollastra-Pisciotto , Cefalù , Italy.
authors: Marco
authors: M
authors: Calvaruso
authors: Alessandro
authors: A
authors: Gulino
authors: Salvatore
authors: S
authors: Buffa
authors: Carla
authors: C
authors: Guarnotta
authors: Giovanni
authors: G
authors: Franco
authors: Matilde
authors: M
authors: Cacciatore
authors: Maria Grazia
authors: MG
authors: Bonura
authors: Vito
authors: V
authors: Franco
authors: Ada Maria
authors: AM
authors: Florena
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D006801:Humans; D008113:Liver Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D016411:Lymphoma, T-Cell, Peripheral / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000378:metabolism*; D013160:Splenic Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D033581:Stem Cell Transplantation / Q000379:methods; D016896:Treatment Outcome
chemical_list: D016692:Receptors, Antigen, T-Cell, gamma-delta
keywords: T cell lymphoma; gamma delta T cell lymphomas; hepatosplenic T cell lymphoma; peripheral T cell lymphomas