Hepatosplenic T-cell lymphoma: A distinct clinicopathologic entity of cytotoxic γδ T-cell origin

C. B. Cooke, L. Krenacs, M. Stetler-Stevenson, T. C. Greiner, M. Raffeld, D. W. Kingma, L. Abruzzo, C. Frantz, M. Kaviani, E. S. Jaffe

Research output: Contribution to journalArticle

286 Scopus citations

Abstract

We identified eight cases of T-cell lymphoma with evidence of a γδ phenotype over a 13-year period. Seven of these cases conformed to a distinct clinicopathologic entity of hepatosplenic γδ T-cell lymphoma. Nearly all of these patients were young adult males (five of seven), with a median age at presentation of 20 years. They presented with marked hepatosplenomegaly, without lymphadenopathy or significant peripheral blood lymphocytosis. Thrombocytopenia was seen in all patients, and five of seven were mildly anemic. The clinical course was aggressive, and despite multiagent chemotherapy, the median survival duration was less than 1 year. The morphologic findings were uniform; a monomorphic population of medium-sized lymphoid cells with moderately clumped chromatin and a rim of pale cytoplasm infiltrated the sinusoids of the spleen, liver, and bone marrow. The cells had a characteristic immunophenotype: CD2+, CD3+, CD4-, CD5-, CD7+, CD16+, CD57-, CD25-, T-cell receptor (TCR)δ+, βF1-. CD8 was positive in four of seven cases tested, and CD56 was positive in five of six. All cases expressed the cytotoxic granule-associated protein, TIA1, but perforin was detected in only one case. All cases with assessable DNA had a TCRγ gene rearrangement, and lacked Epstein-Barr virus sequences. Isochromosome 7q was identified in two cases with cytogenetic information. The one case of cutaneous γδ T-cell lymphoma differed in its clinical manifestations, histologic appearance, and immunophenotype. We conclude that hepatosplenic γδ T-cell lymphoma is a distinct clinicopathologic entity derived from cytotoxic γδ T cells, and should be distinguished from other lymphomas of T-cell and natural-killer cell (NK)-like T-cell derivation.

Original languageEnglish (US)
Pages (from-to)4265-4274
Number of pages10
JournalBlood
Volume88
Issue number11
DOIs
StatePublished - Dec 1 1996

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Cooke, C. B., Krenacs, L., Stetler-Stevenson, M., Greiner, T. C., Raffeld, M., Kingma, D. W., Abruzzo, L., Frantz, C., Kaviani, M., & Jaffe, E. S. (1996). Hepatosplenic T-cell lymphoma: A distinct clinicopathologic entity of cytotoxic γδ T-cell origin. Blood, 88(11), 4265-4274. https://doi.org/10.1182/blood.v88.11.4265.bloodjournal88114265