151. Challenges and new prospects in hepatosplenic γδ T-cell lymphoma
- Author
-
Maria Grazia Bonura, Vito Franco, Giovanni Franco, Salvatore Buffa, Alessandro Gulino, Ada Maria Florena, Carla Guarnotta, Marco Calvaruso, Matilde Cacciatore, Calvaruso, M, Gulino, A, Buffa, S, Guarnotta, C, Franco, G, Cacciatore, M, Bonura MG, Franco, V, and Florena, AM.
- Subjects
Cancer Research ,Hepatosplenic T-cell lymphoma ,Spleen ,Disease ,Biology ,T cell lymphoma ,Antineoplastic Combined Chemotherapy Protocols ,medicine ,Humans ,T-cell lymphoma ,Pathological ,gamma delta T cell lymphoma ,hepatosplenic T cell lymphoma ,Splenic Neoplasms ,Liver Neoplasms ,Lymphoma, T-Cell, Peripheral ,Receptors, Antigen, T-Cell, gamma-delta ,Hematology ,medicine.disease ,Combined Modality Therapy ,peripheral T cell lymphomas ,Lymphoma ,gamma delta T cell lymphomas ,Treatment Outcome ,medicine.anatomical_structure ,Oncology ,Immunology ,Conventional chemotherapy ,Bone marrow ,Stem Cell Transplantation - 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.
- Published
- 2014