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Adolescent Health Medicine

Dubai, UAE
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Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta

Author(s): Farcet JP, Gaulard P, Marolleau JP, Le Couedic JP, Henni T, et al.

Abstract

PeripheralT-celllymphomasconsistofaclinicallyheterogeneousgroupofmalignantdisorderswhoseimmunophenotypeusuallycorrespondstothatofnormalmatureTcells.Wedescribeandcorrelatetheclinical,histopathologic,phenotypic,andgenotypicfindingsintwopatientswithmalignantlymphomapresentingwithhepatosplenicdisease.Themorphologicpatternoflymphomawasthatofasinusal/sinusoidalinfiltrationinspleen,marrow,andliver.ThismorphologiccharacteristicwasassociatedwiththepresenceofaproductiveclonalrearrangementoftheT-cellreceptor(TCR)deltagene.LymphomacellsexpressedaCD3-TCR-gammadelta-phenotype.Theywerealsodoublenegative(ie,CD4-CD8-)andlackedtheCD5andCD7antigens.Inonepatient,tumorprogressionwasassociatedwithphenotypicchangesthatresultedinaCD3-TCR-gammadelta-phenotypewiththesamedelta-generearrangementasinitially.TheseobservationssuggesttheexistenceofanewtypeofperipheralT-celllymphomacharacterizedbyitshepatosplenicpresentation,andbythesinusal/sinusoidaltropismandtheTCR-gammadeltaphenotypeofthemalignantcells.

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