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1、OverviewoftcelllymphomasPadminiMoffett,M.D.DivisionofHematology&MedicalOncologyUniversityofLouisvilleOverviewClassificationofTcellNHLGeneralprinicplesofmanagementMycosisFungoidesPathophysiologyDiagnosisManagementSézarysyndromeGeneralconceptsTcelllymphomascomprise5%-10%ofallNHLMostareaggre
2、ssiveTheiroutcomesareinferiorcomparedtoBcelllymphomasWHOClassificationPrecursorT-cellneoplasmPrecursorT-lymphoblasticlymphoma/leukemiaMature(peripheral)T-cellneoplasmsPredominantlyleukemic/disseminatedT-cellprolymphocyticleukemiaT-cellgranularlymphocyticleukemiaNK-cellleukemiaAdultT-celll
3、ymphoma/leukemia(HTLV1+)PredominantlynodalAngioimmunoblasticT-celllymphomaPeripheralT-celllymphoma,unspecifiedAnaplasticlarge-celllymphoma,T/null-cell,primarysystemictypePredominantlyextranodalMycosisfungoides/SézarysyndromeAnaplasticlarge-celllymphoma,T/null-cell,primarycutaneoustypeExtr
4、anodalNK/T-celllymphoma,nasaltypeEnteropathy-typeT-celllymphomaHepatosplenicT-celllymphomaSubcutaneouspanniculitis-likeT-celllymphomaMature(peripheral)T-cellneoplasmsPredominantlyleukemic/disseminatedAdultT-cellleukemia/lymphomaRetrovirusHTLV-1iscriticaltothedevelopmentofadultT-cellleukem
5、ia/lymphomaEndemicinJapan,theCaribbeanislands,SouthAmerica,andpartsofCentralAfricaTransmittedthroughsexualintercourse,bloodproductscontainingWBCs,sharedneedles,breastmilk,andvertically4clinicalsubtypesAcute—Thesepatientspresentwithsystemicsymptoms,organomegaly,lymphadenopathy,anelevatedla
6、ctatedehydrogenase(LDH)level,andcirculatingmalignantcellsLymphoma-type—ThisvariantischaracterizedbyprominentlymphadenopathywithoutbloodinvolvementChronic—Thesepatientspresentwithskinlesions,mildlymphadenopathy,andleukocytosiswithanabsolutelymphocytosisthatmaybestableformonthstoyearsSmolde
7、ring—Thesepatientsareoftenasymptomaticexceptforfrequentskinand/orpulmonarylesions.Theyhavenormalbloodlymphocytecountswithlessthan5percentcirculatingneoplasticcellsHasproclivityforCNSsoLPmustbepartofinitialstagingTreatmentofATCLLPatientswithtypicalchronicorsmoldering
