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Adolescent Health Medicine

Dubai, UAE
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Hepatosplenicalphabeta T-cell lymphomas: a report of 14 cases and comparison with hepatosplenicgammadelta T-cell lymphomas

Author(s): Macon WR, Levy NB, Kurtin PJ, Salhany KE, Elkhalifa MY, et al.

Abstract

HepatosplenicgammadeltaT-celllymphomaisadistinctentity,characterizedbyoccurrenceinyoungadultmaleswithhepatosplenomegaly,B-symptoms,peripheralbloodcytopenias,andnolymphadenopathy;lymphomatousinfiltratesinthesplenicredpulp,hepaticsinusoids,andbonemarrowsinuses;T-cellreceptor(TCR)gammadeltachainsandacytotoxicT-cellphenotype;isochromosome7q;andanaggressiveclinicalcourse.Incomparison,thisstudydescribestheclinicopathologicfeaturesof14hepatosplenicT-celllymphomasexpressingTCRalphabetachains.Theyoccurredin11womenand3menwithamedianageof36years.ClinicalpresentationwassimilartothatdescribedpreviouslyforhepatosplenicgammadeltaT-celllymphomas,exceptforthefemalepreponderanceandagedistribution(5patientsyoungerthan13yearsofageand5patientsolderthan50yearsofage).Diseasedistributionwasprimarilyinthesplenicredpulpandhepaticsinusoids,althoughliverinfiltrateswerelargelyperiportalinfourcases.Bonemarrowinvolvement,observedineightpatients,wasusuallyinterstitialand/orwithinthesinuses.Lymphnodeswereinvolvedinfivepatients,althoughlymphadenopathywasdemonstrableinonlytwo.Tencaseswerecomposedofintermediate-sizetumorcellswithround/ovalnuclei,slightlydispersedchromatin,inconspicuousnucleoli,andscanttomoderateamountsofcytoplasm.Fourlymphomascontainedprimarilylargecellswithirregularnuclei,dispersedchromatin,discerniblenucleoli,andmoderatetoabundantcytoplasm.Tumorcellsinall14lymphomaswerecytotoxicalphabetaT-cells;13co-expressednaturalkillercell-associatedantigensandshowedT-cellclonality.ThreelymphomaswereassociatedwithEpstein-Barrvirus.Twooffourcaseshadanisochromosome7q.Elevenpatientsaredead,eightwithinayearofdiagnosis,andtwopatientshavemaintainedcompleteremissionsaftercombinationchemotherapy.ThesedatashowthathepatosplenicT-celllymphomasincludeanalphabeta-subtype.Thisgroup,alongwiththepreviouslyrecognizedgammadeltagroup,shouldberecognizedasphenotypicallyheterogeneoussubtypesofthesamediseaseentity.

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