Hepatosplenic gammadelta T-cell lymphoma: ultrastructural, immunophenotypic, and functional evidence for cytotoxic T lymphocyte differentiation. Human pathology. [Hum Pathol] Journal article | | Title | Hepatosplenic gammadelta T-cell lymphoma: ultrastructural, immunophenotypic, and functional evidence for cytotoxic T lymphocyte differentiation. | | Author(s) | Salhany KE, Feldman M, Kahn MJ, Peritt D, Schretzenmair RD, Wilson DM, DiPaola RS, Glick AD, Kant JA, Nowell PC, Kamoun M | | Institution | Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine and Wistar Institute Philadelphia 19104, USA. | | Source | Hum Pathol 1997 Jun; 28(6):674-85. | | MeSH | Adolescent
Adult
Aged
Animals
Guinea Pigs
Humans
Immunophenotyping
Interferon Type II
Interleukin-4
Liver Neoplasms
Lymphoma, T-Cell
Male
Membrane Glycoproteins
Membrane Proteins
Middle Aged
Proteins
RNA-Binding Proteins
Receptors, Antigen, T-Cell, gamma-delta
Research Support, Non-U.S. Gov't
Serine Endopeptidases
Splenic Neoplasms
T-Lymphocytes, Cytotoxic
| | Abstract | Hepatosplenic gammadelta T cell lymphoma (TCL) is a rare, aggressive subset of peripheral TCL that presents with hepatosplenomegaly and cytopenias. Detailed clinicopathological, ultrastructural, and cytogenetic analyses of these lymphomas are limited; functional characteristics of these lymphomas are unknown. We have undertaken a clinicopathological, immunophenotypic, ultrastructural, cytogenetic, and functional analysis of three hepatosplenic gammadelta TCLs. All patients presented with massive hepatosplenomegaly and anemia, thrombocytopenia, or severe neutropenia; terminal blastlike transformation occurred in one patient. Combination chemotherapy had no response in two patients, but induced complete remission in one. gammadelta T cell receptor (TCR) expression and clonal TCRdelta gene rearrangements were documented in each case. Two different subsets of gammadelta TCL were identified based on delta chain variable region usage; two lymphomas were Vdelta1+, whereas the third was negative for both Vdelta1 and Vdelta2. Cytogenetic analysis was performed on two lymphomas; isochromosome 7q and probable trisomy 8 was shown in one of the Vdelta1+ lymphomas, whereas the Vdelta1 negative lymphoma had 14p+ with t(1;14)(q21;p13). NK cell-associated antigens (CD11c, CD16, or CD56) and cytotoxic T lymphocyte (CTL) effector proteins (perforin, granzyme B, TIA-1, and Fas ligand) were expressed by each lymphoma; dense core cytolytic granules were observed by electron microscopy in both lymphomas studied. Functional studies performed in two cases showed TCR-mediated cytolysis of P815 x 2 FcR+ cells induced by anti-CD3 in a redirected cytolysis assay in one of the CD56+, Vdelta1+ lymphomas, whereas IFNgamma secretion was induced by anti-CD3 in the CD56-, Vdelta1 negative lymphoma. These studies show that hepatosplenic gammadelta TCLs have CTL differentiation, retain functional activity in vitro, and are derived from at least two gammadelta T cell subsets. | | Language | eng | | Pub Type(s) | Journal Article
| | PubMed ID | 9191001 |
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