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Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of
cytotoxic gamma delta T-cell origin
CB Cooke, L Krenacs, M Stetler-Stevenson, TC Greiner, M Raffeld, DW Kingma, L Abruzzo, C Frantz, M Kaviani and ES Jaffe
Hematopathology Section, National Cancer Institute, National Institutes of
Health, Bethesda, MD 20892-1500, USA.
We identified eight cases of T-cell lymphoma with evidence of a gamma delta
phenotype over a 13-year period. Seven of these cases conformed to a
distinct clinicopathologic entity of hepatosplenic gamma delta 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)delta +,
beta F1-. 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 TCR gamma gene rearrangement, and
lacked Epstein-Barr virus sequences. Isochromosome 7q was identified in two
cases with cytogenetic information. The one case of cutaneous gamma delta
T-cell lymphoma differed in its clinical manifestations, histologic
appearance, and immunophenotype. We conclude that hepatosplenic gamma delta
T-cell lymphoma is a distinct clinicopathologic entity derived from
cytotoxic gamma delta T cells, and should be distinguished from other
lymphomas of T-cell and natural-killer cell (NK)-like T-cell derivation.
Volume 88,
Issue 11,
pp. 4265-4274,
12/01/1996
Copyright © 1996 by The American Society of Hematology

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