6533b7d3fe1ef96bd1260aa4
RESEARCH PRODUCT
Challenges and new prospects in hepatosplenic γδ T-cell lymphoma.
Maria Grazia BonuraVito FrancoGiovanni FrancoSalvatore BuffaAlessandro GulinoAda Maria FlorenaCarla GuarnottaMarco CalvarusoMatilde Cacciatoresubject
Cancer ResearchHepatosplenic T-cell lymphomaSpleenDiseaseBiologyT cell lymphomaAntineoplastic Combined Chemotherapy ProtocolsmedicineHumansT-cell lymphomaPathologicalgamma delta T cell lymphomahepatosplenic T cell lymphomaSplenic NeoplasmsLiver NeoplasmsLymphoma T-Cell PeripheralReceptors Antigen T-Cell gamma-deltaHematologymedicine.diseaseCombined Modality Therapyperipheral T cell lymphomasLymphomaT cell lymphoma; gamma delta T cell lymphomas; hepatosplenic T cell lymphoma; peripheral T cell lymphomasTreatment Outcomemedicine.anatomical_structureOncologyImmunologyConventional chemotherapyBone marrowStem Cell Transplantationdescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2014-03-10 |