Hepatosplenic and other γδ T-cell lymphomas

Francisco Vega, L. Jeffrey Medeiros, Philippe Gaulard

Research output: Contribution to journalArticle

104 Scopus citations

Abstract

The 2005 Society for Hematopathology/European Association for Haematopathology Workshop session 11 was dedicated to hepatosplenic T-cell lymphoma (HSTCL). HSTCL is a rare aggressive type of extranodal lymphoma characterized by hepatosplenomegaly, bone marrow involvement, and peripheral blood cytopenias. HSTCL exhibits a distinctive pattern of infiltration; tumor cells preferentially infiltrate the sinusoids of the splenic red pulp, liver, and bone marrow. The tumor cells have a nonactivated cytotoxic T-cell immunophenotype and frequently carry a recurrent cytogenetic abnormality, isochromosome 7q. Most cases express the γδ T-cell receptor, but cases can have an αβ phenotype and are considered to be a variant of the disease. Although HSTCL is the prototype peripheral T-cell lymphoma expressing the γδ T-cell receptor, non-HSTCL proliferations of γδ T cells can involve other extranodal sites, mainly skin and mucosa. These γδ T-cell lymphomas display marked heterogeneity in clinical and histologic features. In contrast with HSTCL, non-HSTCL γδ T-cell lymphomas frequently have an activated cytotoxic phenotype and most likely are not a single disease entity.

Original languageEnglish (US)
Pages (from-to)869-880
Number of pages12
JournalAmerican journal of clinical pathology
Volume127
Issue number6
DOIs
StatePublished - Jun 1 2007
Externally publishedYes

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Keywords

  • γδ T-cell lymphoma
  • Hepatosplenic T-cell lymphoma
  • T-cell neoplasms
  • WHO classification
  • Workshop 2005, Houston

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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