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Adolescent Health Medicine

Dubai, UAE
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Hepatosplenicgammadelta 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, et al.

Abstract

HepatosplenicgammadeltaTcelllymphoma(TCL)isarare,aggressivesubsetofperipheralTCLthatpresentswithhepatosplenomegalyandcytopenias.Detailedclinicopathological,ultrastructural,andcytogeneticanalysesoftheselymphomasarelimited;functionalcharacteristicsoftheselymphomasareunknown.Wehaveundertakenaclinicopathological,immunophenotypic,ultrastructural,cytogenetic,andfunctionalanalysisofthreehepatosplenicgammadeltaTCLs.Allpatientspresentedwithmassivehepatosplenomegalyandanemia,thrombocytopenia,orsevereneutropenia;terminalblastliketransformationoccurredinonepatient.Combinationchemotherapyhadnoresponseintwopatients,butinducedcompleteremissioninone.gammadeltaTcellreceptor(TCR)expressionandclonalTCRdeltagenerearrangementsweredocumentedineachcase.TwodifferentsubsetsofgammadeltaTCLwereidentifiedbasedondeltachainvariableregionusage;twolymphomaswereVdelta1+,whereasthethirdwasnegativeforbothVdelta1andVdelta2.Cytogeneticanalysiswasperformedontwolymphomas;isochromosome7qandprobabletrisomy8wasshowninoneoftheVdelta1+lymphomas,whereastheVdelta1negativelymphomahad14p+witht(1;14)(q21;p13).NKcell-associatedantigens(CD11c,CD16,orCD56)andcytotoxicTlymphocyte(CTL)effectorproteins(perforin,granzymeB,TIA-1,andFasligand)wereexpressedbyeachlymphoma;densecorecytolyticgranuleswereobservedbyelectronmicroscopyinbothlymphomasstudied.FunctionalstudiesperformedintwocasesshowedTCR-mediatedcytolysisofP815x2FcR+cellsinducedbyanti-CD3inaredirectedcytolysisassayinoneoftheCD56+,Vdelta1+lymphomas,whereasIFNgammasecretionwasinducedbyanti-CD3intheCD56-,Vdelta1negativelymphoma.ThesestudiesshowthathepatosplenicgammadeltaTCLshaveCTLdifferentiation,retainfunctionalactivityinvitro,andarederivedfromatleasttwogammadeltaTcellsubsets.

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