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Adolescent Health Medicine

Dubai, UAE
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Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin

Author(s): Cooke CB, Krenacs L, Stetler-Stevenson M, Greiner TC, Raffeld M, et al.

Abstract

WeidentifiedeightcasesofT-celllymphomawithevidenceofagammadeltaphenotypeovera13-yearperiod.SevenofthesecasesconformedtoadistinctclinicopathologicentityofhepatosplenicgammadeltaT-celllymphoma.Nearlyallofthesepatientswereyoungadultmales(fiveofseven),withamedianageatpresentationof20years.Theypresentedwithmarkedhepatosplenomegaly,withoutlymphadenopathyorsignificantperipheralbloodlymphocytosis.Thrombocytopeniawasseeninallpatients,andfiveofsevenweremildlyanemic.Theclinicalcoursewasaggressive,anddespitemultiagentchemotherapy,themediansurvivaldurationwaslessthan1year.Themorphologicfindingswereuniform;amonomorphicpopulationofmedium-sizedlymphoidcellswithmoderatelyclumpedchromatinandarimofpalecytoplasminfiltratedthesinusoidsofthespleen,liver,andbonemarrow.Thecellshadacharacteristicimmunophenotype:CD2+,CD3+,CD4-,CD5-,CD7+,CD16+,CD57-,CD25-,T-cellreceptor(TCR)delta+,betaF1-.CD8waspositiveinfourofsevencasestested,andCD56waspositiveinfiveofsix.Allcasesexpressedthecytotoxicgranule-associatedprotein,TIA1,butperforinwasdetectedinonlyonecase.AllcaseswithassessableDNAhadaTCRgammagenerearrangement,andlackedEpstein-Barrvirussequences.Isochromosome7qwasidentifiedintwocaseswithcytogeneticinformation.TheonecaseofcutaneousgammadeltaT-celllymphomadifferedinitsclinicalmanifestations,histologicappearance,andimmunophenotype.WeconcludethathepatosplenicgammadeltaT-celllymphomaisadistinctclinicopathologicentityderivedfromcytotoxicgammadeltaTcells,andshouldbedistinguishedfromotherlymphomasofT-cellandnatural-killercell(NK)-likeT-cellderivation.

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