Gamma-delta t-cell lymphomas

Marco Foppoli, Andrés J M Ferreri

Research output: Contribution to journalArticle

Abstract

Gamma-delta T-cell lymphomas are aggressive and rare diseases originating from gamma-delta lymphocytes. These cells, which naturally play a role in the innate, non-specific immune response, develop from thymic precursor in the bone marrow, lack the major histocompatibility complex restrictions and can be divided into two subpopulations: Vdelta1, mostly represented in the intestine, and Vdelta2, prevalently located in the skin, tonsils and lymph nodes. Chronic immunosuppression such as in solid organ transplanted subjects and prolonged antigenic exposure are probably the strongest risk factors for the triggering of lymphomagenesis. Two entities are recognised by the 2008 WHO Classification: hepatosplenic gamma-delta T-cell lymphoma (HSGDTL) and primary cutaneous gamma-delta T-cell lymphoma (PCGDTL). The former is more common among young males, presenting with B symptoms, splenomegaly and thrombocytopenia, usually with the absence of nodal involvement. Natural behaviour of HSGDTL is characterised by low response rates, poor treatment tolerability, common early progression of disease and disappointing survival figures. PCGDTL accounts for

Original languageEnglish
Pages (from-to)206-218
Number of pages13
JournalEuropean Journal of Haematology
Volume94
Issue number3
DOIs
Publication statusPublished - Mar 1 2015

Keywords

  • Extranodal lymphoma
  • Gamma-delta T lymphocytes
  • Hepatosplenic gamma-delta T-cell lymphoma
  • Primary cutaneous gamma-delta T-cell lymphoma
  • T-cell lymphoma

ASJC Scopus subject areas

  • Hematology
  • Medicine(all)

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