摘要：

Hepatosplenic gammadelta T () is a rare, aggressive subset of peripheral that presents with hepatosplenomegaly and . Detailed clinicopathological, ultrastructural, and cytogenetic analyses of these are limited; functional characteristics of these are unknown. We have undertaken a clinicopathological, immunophenotypic, ultrastructural, cytogenetic, and functional analysis of three hepatosplenic gammadelta . All patients presented with massive hepatosplenomegaly and , thrombocytopenia, or severe ; terminal blastlike transformation occurred in one patient. Combination chemotherapy had no response in two patients, but induced complete remission in one. gammadelta T receptor (TCR) expression and clonal TCRdelta gene rearrangements were documented in each case. Two different subsets of gammadelta were identified based on chain variable region usage; two were Vdelta1+, whereas the third was negative for both Vdelta1 and Vdelta2. Cytogenetic analysis was performed on two ; isochromosome 7q and probable 8 was shown in one of the Vdelta1+ , whereas the Vdelta1 negative had 14p+ with t(1;14)(q21;). NK antigens (CD11c, CD16, or CD56) and cytotoxic T lymphocyte () effector (perforin, , , and ) were expressed by each ; dense core were observed by microscopy in both studied. Functional studies performed in two cases showed TCR-mediated of P815 x 2 FcR+ induced by anti-CD3 in a redirected assay in one of the CD56+, Vdelta1+ , whereas was induced by anti-CD3 in the CD56-, Vdelta1 negative . These studies show that hepatosplenic gammadelta have differentiation, retain functional activity in vitro, and are derived from at least two gammadelta T subsets.

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