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Adolescent Health Medicine

Dubai, UAE
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Hepatosplenicalphabeta T-cell lymphoma with myelodysplastic syndrome

Author(s): Takaku T, Miyazawa K, Sashida G, Shoji N, Shimamoto T, et al.

Abstract

Wedescribeapatientwithhepatosplenic33T-celllymphomawhoshowedpancytopeniaandmyelodysplasia.A35-year-oldmanwasadmittedwithfever,pancytopenia,andhepatosplenomegalybutwithnolymphadenopathy.Wealsofoundtrilineagemyelodysplasiainthebonemarrowonhisfirstadmission.Thepatienthadhighfeverandanemiabutnoevidenceofinfectionandwastentativelytreatedwithprednisolone.Thistreatmentresultedinatransientimprovementofthecytopeniaandareductionofspleensize.However,10monthsafterthefirstmanifestation,progressionofthesplenomegalyandfeverbecameapparent,andasplenectomywasperformed.Thepathologicfindingsforthespleenshoweddiffuseanddisseminatedinfiltrationofmedium-tolarge-sizedT-lymphocytesinthesplenicredpulp.ThesecellswereimmunohistochemicallypositiveforCD3,CD5,CD7,CD8,CD16,CD56,T-cellreceptor33(TCR33),T-cellintracellularantigen1,andgranzymeBbutwerenegativeforCD4,CD30,CD57,andTCR33.Thesedatasuggestedadiagnosisofhepatosplenic33T-celllymphoma.ASouthernblotanalysisrevealedgenerearrangementoftheTCR3-chaingenebutnottheimmunoglobulinheavychaingeneinthespleencells.AninsituhybridizationanalysisfortheEpstein-Barrvirusrevealednegativeresults.Thepatientreceived8coursesofcombinationchemotherapyandachievedapartialremission;however,thedysplasticfeaturesofthemarrowcellspersistedafterthepartialremissionwasobtained.Additionaltreatmentwithallogeneicbonemarrowtransplantationresultedinatransientcompleteremission;however,thepatientrelapsed11monthslater.Becausehehadexperiencednolymphadenopathyandshoweddysplasticfeaturesinthebonemarrow,thediagnosiswashighlydependentonthepathologicfindingsfortheresectedspleen.

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