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Hepatosplenic and Other γΔ T-Cell Lymphomas

Francisco Vega MD, PhD, L. Jeffrey Medeiros MD, Philippe Gaulard MD
DOI: http://dx.doi.org/10.1309/LRKX8CE7GVPCR1FT 869-880 First published online: 1 June 2007

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.

Key Words:
  • Hepatosplenic T-cell lymphoma
  • γΔ T-cell lymphoma
  • WHO classification
  • T-cell neoplasms
  • Workshop 2005, Houston