T细胞淋巴瘤2022.v1（英文）-NCCN肿瘤临床实践指南

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)T-CellLymphomasionMarchNCCNGuidelinesforPatients®availableat/patientsVersion2.2022,03/07/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20226:59:41AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dex*StevenM.Horwitz,MD/Chair†ÞMemorialSloanKetteringCancerCenter*StephenAnsell,MD,PhD/Vice-Chair‡MayoClinicCancerCenterWeiyunZ.Ai,MD,PhD†‡UCSFHelenDillerFamilyComprehensiveCancerCenterJeffreyBarnes,MD,PhD†MassachusettsGeneralHospitalCancerCenter*StefanK.Barta,MD,MRCP,MS†‡AbramsonCancerCenterheUniversityofPennsylvania*JonathanBrammer,MD‡TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstitute*MarkW.Clemens,MDʘTheUniversityofTexasAhmetDogan,MD,PhD≠MemorialSloanKetteringCancerCenterFrancineFoss,MD†‡ξYaleCancerCenter/SmilowCancerHospital*PaolaGhione,MD‡RoswellParkComprehensiveCancerCenter*AaronM.Goodman,MD‡ξUCSanDiegoMooresCancerCenterJoanGuitart,MD≠ϖRobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityesPanelDisclosures*AhmadHalwani,MD‡HuntsmanCancerInstitute*AhmadHalwani,MD‡HuntsmanCancerInstituteattheUniversityofUtah*BradleyM.Haverkos,MD,MPH,MS†UniversityofWisconsinCarboneCancerCenterSimaRozati,MD,PhDϖofColoradoCancerCenterRichardT.Hoppe,MDofColoradoCancerCenterRichardT.Hoppe,MD§StanfordCancerInstituteJacobsenMD*JonathanSaid,MD≠UCLAJonssonComprehensiveCancerCentermandWomensentermandWomensenter*DeepaJagadeesh,MD,MPH†‡CaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussignstituteAllisonJones,MDϖSt.JudeChildren'sResearchHospital/TheUniversityofTennesseeHealthScienceCenterAvyaktaKallam,MD,MBBSÞVanderbilt-IngramCancerCenter*LaurenShea,MD‡O'NealComprehensiveCancerCenteratUAB*MichiM.Shinohara,MDϖ≠FredHutchinsonCancerResearchCenter/SeattleCancerCareAlliance*LubomirSokol,MD,PhD†‡ÞMoffittCancerCenterCarlosTorres-Cabala,MD≠TheUniversityofTexasfettfettCancerCenterYounH.Kim,MDϖ†StanfordCancerInstituteKiranKumar,MD,MBA§UTSouthwesternSimmonsComprehensiveCancerCenter*NehaMehta-Shah,MD,MSCI†‡*RyanWilcox,MD,PhD†‡UniversityofMichiganRogelCancerCenter*PeggyWu,MD,MPHϖUCDavisComprehensiveCancerCenterJasmineZain,MD†‡nCancerCenteratBarnesCitynCancerCenteratBarnesalandWashingtonoolofMedicineEliseA.Olsen,MDϖ†DukeCancerInstituteξBonemarrowtransplantation≠PathologyϖDermatologyʘPlasticsurgery‡Hematology/Hematology§Radiotherapy/RadiationoncologyoncologyÞInternalmedicine*DiscussionWriting†MedicaloncologyCommitteeMemberVersion2.2022,03/07/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20226:59:41AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexelievesthattheelievesthattheanagementforanypatientwitherisinaclinicaltrialipationinclinicaltrialsisespeciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions.ofEvidenceandndationsareseindicatedNCategoriesofEvidenceandConsensus.NCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.yoftheGuidelinesUpdates•PeripheralT-CellLymphomas(TCEL-1)•BreastImplant-AssociatedAnaplasticLargeCellLymphoma(BIAA-INTRO)•T-CellLargeGranularLymphocyticLeukemia(LGLL-INTRO)•T-CellProlymphocyticLeukemia(TPLL-1)•AdultT-CellLeukemia/Lymphoma(ATLL-1)•HepatosplenicT-CellLymphoma(HSTCL-INTRO)•ExtranodalNK/T-CellLymphomas(NKTL-1)•PrinciplesofMolecularAnalysisinT-CellLymphomas(TCLYM-A)•SupportiveCare(TCLYM-B)•LuganoResponseCriteriaforNon-HodgkinLymphoma(TCLYM-C)•PrinciplesofRadiationTherapy(TCLYM-D)•SeeNCCNGuidelinesforPrimaryCutaneousLymphomaspPrimaryCutaneousB-CellLymphomaspMycosisFungoides/SézarySyndromepPrimaryCutaneousCD30+T-CellLymphoproliferativeDisordersingSTDiagnosisofMatureBCellmsSeeNCCNGuidelinesforBCellLymphomasNHODGATheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2022.Version2.2022,03/07/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20226:59:41AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexUpdatesinVersion2.2022oftheNCCNGuidelinesforT-CellLymphomasfromVersion1.2022include:MS-1•TheDiscussionsectionhasbeenupdatedtoreflectthechangesinthealgorithm.UpdatesinVersion1.2022oftheNCCNGuidelinesforT-CellLymphomasfromVersion1.2021include:Globalchanges•Suggestedtreatmentregimenreferenceswereupdatedthroughouttheguidelines.•Diagnosis,molecularandIHCtestingwereclarifiedas"mayinclude"asappropriate.PeripheralT-CellLymphomasL•DiagnosispEssential,3rdbullet,1stsub-bullet:EBER-ISHwasremovedandaddedasaseparatebullet.(alsoforHSTCL-1andNKTL-1)pUseful,3rdbulletrevised:...cellorigin(CXCL13,ICOS,PD1),and...L•Afterfirst-linetherapy,algorithmrevisedbymovingfootnote,"ConsiderconsolidativeHDT/ASCRforhigh-riskIPIpatientsinCR1"intothealgorithmflow.End-oftreatmentstagingandfollow-uprecommendationswereadded.•Footnotelrevised:andtreatmentaccordingtotheALCL,ALK-positivealgorithmmaybeconsideredforALK-negativeALCLwithDUSP22rearrangementTCEL-4andTCEL-5•BothALCL,ALK-positiveandOtherhistologies,InterimRestagingandAdditionalTherapy:Follow-uprecommendationswereadded.L•Follow-uprecommendationswereaddedaftercompletionoftreatmentforrelapsed/refractorydisease.TCEL-B2of7•Initialpalliativeintenttherapy,suggestedtreatmentregimensforPTCL-NOS;EATL;MEITLandAITL,includingNodalPTCL,TFHandFTCLandAITLmovedtosamepage.pDuvelisibaddedasacategory2A,otherrecommendedregimenforallsubtypes.Correspondingfootnotekadded:InthephaseIIstudy,thepreferreddosingregimenofduvelisibwas75mgfor2cyclesfollowedby25mgBIDforlong-termdiseasecontrol.pAlectinibaddedasacategory2A,otherrecommendedregimenforALK+ALCLonly.Correspondingfootnoteoadded:AlectinibhasshownactivityinpatientswithCNSinvolvement.TCEL-B3of7andTCEL-B5of7•Second-linetherapyandsubsequenttherapyforPTCL-NOS;EATL;MEITandALCLpSubtype,NodalPTCL,TFHandFTCLmovedtoAITLpageofrecommendations.pDuvelisibaddedasacategory2A,otherrecommendedregimenforbothintentiontoproceedtotransplantandnointentiontotransplant.Correspondingfootnotekadded:InthephaseIIstudy,thepreferreddosingregimenofduvelisibwas75mgfor2cyclesfollowedby25mgBIDforlong-termdiseasecontrol.pAlectinibaddedasacategory2A,otherrecommendedregimenforALK+ALCLonly.Correspondingfootnoteoadded:AlectinibhasshownactivityinpatientswithCNSinvolvement.TCEL-B4of7•Second-linetherapyandsubsequenttherapyforAITL,INCLUDINGNODALPTCL,TFHandFTCLpDuvelisibaddedasacategory2A,otherrecommendedregimenforbothintentiontoproceedtotransplantandnointentiontotransplant.Correspondingfootnotekadded:InthephaseIIstudy,thepreferreddosingregimenofduvelisibwas75mgfor2cyclesfollowedby25mgBIDforlong-termdiseasecontrol.ContinuedVersion2.2022,03/07/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESPrintedbyMinTangon3/14/20226:59:41AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexuidelinesforTCellLymphomasfromVersionincludeatedALCL•Treatment,2ndbulletrevised:ConsiderRemovalofcontralateralimplant•Follow-upwasrevisedfrom"C/A/PCTwithcontrastorPET/CTscanasclinicallyindicated"to"Surveillanceimagingnomoreoftenthanevery6mofor2yandthenannuallyfor5yorasclinicallyindicated"T-CellLargeGranularLymphocyticLeukemiaLGLL-INTRO•1stbulletrevisedbyremoving:SomeinvestigatorsregardLGLleukemiaasaclonallymphoproliferationofunknownsignificanceratherthanaleukemia.LGLL-1•DiagnosispEssential,2ndbulletrevisedbyremovingsub-bullet:IHCpanel:CD3,CD4,CD5,CD7,CD8,CD56,CDLGLL-2•Footnotemrevisedfrom"Forlong-termusewitheithermethotrexateorcyclophosphamide,monitoringforcumulativetoxicityisrecommended"to"Monitoringforcumulativetoxicityisrecommendedforlong-termusewithmethotrexate."phocyticLeukemiaL•DiagnosispEssential,2ndbulletrevised:Peripheralbloodflowcytometrywithadequateimmunophenotypingtoestablishdiagnosis,Cellsurfacemarkeranalysismayinclude:...pUseful,2ndbulletrevised:BonemarrowaspirateandbiopsyLompleteorpartialresponseaddedaqualifierafterconsiderallogeneicHCTRelapseorProgressivediseaseanddirectedthealgorithmtothesecondlinetherapyrecommendations.emiaLymphoma•Diagnosis,Essentialp2ndbulletrevisedfrom"Flowcytometryonperipheralblood"to"Peripheralbloodflowcytometrywithadequateimmunophenotypingtoestablishdiagnosis"p3rdbulletadded:Cellsurfacemarkeranalysisbyflowcytometrymayinclude:CD2,CD3,CD4,CD5,CD7,CD8,CD25,CD30,TCRαß•Workup,Usefulp4thbulletadded:EchocardiogramorMUGAscanifanthracycline-basedregimenisindicatedpBulletremoved:SkeletalsurveyinsymptomaticpatientsATLL-C1of2•Footnotedrevisedbyremoving,"Aretrospectivestudyshowedaparticularlyhighriskofdevelopinggraft-versushostdisease(GVHD)inpatientsproceedingtoallogenictransplantwithin50daysofmogamulizumab(FujiS,etal.JClinOncol2016;34:3426-3433)"andaddingthistotheSupportiveCarepage,TCLYM-B3of3UPDATESVersion2.2022,03/07/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion2.2022,03/07/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20226:59:41AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexuidelinesforTCellLymphomasfromVersionincludeHepatosplenicT-CellLymphomaHSTCL-1•Diagnosis,Usefulp4thbulletrevisedfrom"Genomicanalysisfor..."to"Next-generationsequencing(NGS)panelmayinclude..."HSTCL-A•SuggestedtreatmentregimenspMovedregimensfromOtherrecommendedregimenstoUsefulincertaincircumstances◊Alemtuzumab+pentostatin◊CHOEP◊Dose-adjustedEPOCHpMovedregimensfromUsefulincertaincircumstancestoOtherrecommendedregimens◊DHAP(dexamethasone,cytarabine,cisplatin)◊DHAX(dexamethasone,cytarabine,oxaliplatin)pUsefulincertaincircumstances,Brentuximabvedotin+CHP(cyclophosphamide,doxorubicin,andprednisone)forCD30+caseswaschangedfromacategory2Atoacategory2Brecommendation.ExtranodalNK/T-CellLymphomas•AlgorithmnamechangedfromExtranodalNK/T-CellLymphoma,nasaltypetoExtranodalNK/T-CellLymphomas.•Workup,Essential,13thbulletrevised:EchocardiogramorMUGAscaniftreatmentincludesregimenscontaininganthracyclinesoranthracenedioneanthracycline-basedregimenisindicated•Footnoteadded:Itispreferredthattreatmentoccuratcenterswithexpertiseinthemanagementofthisdisease.gativeafterabiopsyoptionrevisedConsiderHCTifeligibleFootnotedaddedAsparaginaseErwiniachrysanthemicanbesubstitutedforpegaspargaseinpatientswithsystemicallergicreactionoranaphylaxisdueopegaspargasehypersensitivityPrintedbyMinTangon3/14/20226:59:41AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexuidelinesforTCellLymphomasfromVersionincludeSupportiveCareTYLYM-B1of3•TreatmentofTLS,First-lineandatretreatmentforhyperuricemiarevised:pGlucose-6-phosphatedehydrogenase(G6PD)testingisrequiredpriortouseofrasburicase.RasburicaseiscontraindicatedinpatientswithahistoryconsistentwithG6PD.Inthesepatients,rasburicaseshouldbesubstitutedwithallopurinol.◊LowRiskDisease:Allopurinolorfebuxostatifintoleranttoallopurinolbeginning2–3dayspriortochemoimmunotherapyandcontinuedfor10–14daysor◊te:StageI/IIandLDH<2XULN:Rasburicaseifrenaldysfunctionanduricacid,potassium,and/orphosphate>ULNTCLYM-B3of3•MogamulizumabandDrugEruptionbulletadded:MogamulizumabhasbeenassociatedwithadrugeruptionthatcanclinicallymimiccutaneousT-celllymphoma.Skinbiopsyisrecommendedtodistinguishprogressionofdiseaseversusdrugeruption(ChenL,etalJAMADermatology2019;155:968-971;HirotsuK,etalJAMADermatol2021;157:700-707).PrinciplesofRadiationTherapyTCLYM-D1of4•Bulletaddedwithalink:SeeNCCNGuidelinesforHodgkinLymphoma-RadiationDoseConstraints.ReviewofallslideswithatleastoneparaffinblockrepresentativeofthetumorshouldbedonebyahematopathologistwithexpertiseinthediagnosisofPTCL.Rebiopsyifconsultmaterialisnondiagnostic.•Excisionalorincisionalbiopsyispreferredovercoreneedlebiopsy.AfineneedleReviewofallslideswithatleastoneparaffinblockrepresentativeofthetumorshouldbedonebyahematopathologistwithexpertiseinthediagnosisofPTCL.Rebiopsyifconsultmaterialisnondiagnostic.•Excisionalorincisionalbiopsyispreferredovercoreneedlebiopsy.AfineneedleaspirationFNAbiopsyaloneisnotsufficientfortheinitialosisoflymphomaAcoreneedlebiopsyisnotoptimalbutcanbeusedundercertaincircumstancesIncertaincircumstanceswhenanodeisnoteasilyaccessibleforexcisionalorincisionalbiopsyinationofcoreneedlebiopsyandFNAbiopsyinconjunctionwithappropriateancillarytechniquesmaybesufficientfordiagnosis.dequateimmunophenotypingtoestablishdiagnosisbsidermolecularanalysistodetectDUSPrearrangementifALCLALKnegativea;TP63rearrangementifIHCispositiveforTP63•AdditionalimmunohistochemicalstudiestocharacterizesubsetsofkersofTfollicularhelperTFHcelloriginCXCLndcytotoxicTcellmarkersTIAgranzymeBperforinntofHTLVdbyserologyorothermethodsisencouragedsultscanimpacttherapyALCLALKnegative(TCEL-2)•Enteropathy-associatedT-celllymphoma(EATL)•MonomorphicepitheliotropicintestinalT-cellg•NodalperipheralT-celllymphomawithTFHphenotype(NodalPTCL,TFH)•FollicularT-celllymphoma(FTCL)llymphomasNTROpExtranodalnaturalkiller(NK)/T-celllymphoma,nasaltype(ENKL)(SeeNKTL-1)pHepatosplenicT-celllymphoma(HSTCL)(SeeHSTCL-INTRO)Subtypesnotincluded:lambdaCDCDCDCDCD10,CD20,CD30,CD4,CD8,CD7,BCLKiCDCDCD,CD4,CD8,CD7,CD56,CD21,CD23,dexESSENTIAL:TCRβ,TCRẟ,PD1/CD279,ALK,TP63DTCRTCREBER-ISHUSEFULUNDERCERTAINCIRCUMSTANCES:entsorotherassessmentofclonalityc•MolecularanalysistodetectclonalT-entsorotherassessmentofclonalitycSSubtypesincluded:e•PeripheralT-celllymphoma(PTCL),nototherwisemmunoblasticTmmunoblasticTcelllymphomaAITLflymphomakinase(ALK)positiveWorkup•Anaplasticlargecelllymphomakinase(ALK)positiveWorkuputaneousALCLSeeNCCNGuidelinesformascClonalTCRgenerearrangementsalonearenotsufficientfordiagnosis,asthesecanalsobeseeninpatientswithnon-malignantconditions.Resultsshouldbelnit-icClonalTCRgenerearrangementsalonearenotsufficientfordiagnosis,asthesecanalsobeseeninpatientswithnon-malignantconditions.ResultsshouldbetofoverallpresentationSeePrinciplesofMolecularAnalysisinTCellLymphomasTCLYMAandappropriateIHCshouldbeperformedClonaltofoverallpresentationSeePrinciplesofMolecularAnalysisinTCellLymphomasTCLYMANote:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version2.2022,03/07/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.TCEL-1•CalculationofInternationalPrognosticIndex(IPI)ihocardiogramorMUGAscanifanthracyclinebasedregimenisindicatedPregnancytestinginpatientsofchildbearingpotential(ifchemotherapyorRTis•CalculationofInternationalPrognosticIndex(IPI)ihocardiogramorMUGAscanifanthracyclinebasedregimenisindicatedPregnancytestinginpatientsofchildbearingpotential(ifchemotherapyorRTisDiscussionoffertilityissuesandspermbankingdexORKUPS•Historyandphysical(H&P)examination;fullskinexamination;attentiontonode-bearingareas,includingWaldeyer'sring;evaluationofsizeofliverandspleen,nasopharynx•Performancestatus•Bsymptoms•CBCwithdifferential•Bonemarrowbiopsy±aspirate•Lactatedehydrogenase(LDH)•Comprehensivemetabolicpanel•PET/CT•PET/CTscanh(preferred)and/orchest/abdominal/pelvic(C/A/P)CTwithcontrastgnosticqualityDCASES•NeckCTwithcontrastConsiderCNSevaluationifclinicalsigns/symptomsConsiderCNSevaluationifclinicalsigns/symptomsj•Skinbiopsy•HIVtesting•HepatitisBandCtesting•ConsiderquantitativeEBVpolymerasechainreaction(PCR)•ConsiderceliacdiseaseinnewlydiagnosedEATLlydoneasresultscanimpacttherapyd•AssessmentofHTLV-1/2byserologylydoneasresultscanimpacttherapydALCL,ALKpositiveSeeTCEL-3ALCL,ALKnegativeAITLMEITLNodalPTCL,TFHFTCLSeeTCEL-3dSeemapforprevalenceofHTLV-1/2bygeographicregion.HTLV-1/2hasbeendescribedinpatientsinnon-endemicareas.hPatientswithT-celllymphomasoftenhaveextranodaldisease,whichmaybeinadequatelyimagedbyCT.PETscanispreferred.iSeeInternationalPrognosticIndex(TCEL-A).jTheroleofintrathecalprophylaxisinPTCLislargelyunknown.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version2.2022,03/07/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.TCEL-2PrintedbyMinTangon3/14/20226:59:41AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexSUBTYPEkSTAGEFIRST-LINETHERAPYINTERIMRESTAGINGrtumorlysissyndromeSeeTCLYMBALCL,ALKpositiveStageI,IIStageIII,IVMultiagentchemotherapymx6cycles±involved-siteRT(ISRT)norMultiagentchemotherapymx3–4cycles+ISRTn(category2B)Multiagentchemotherapymx6cyclesthththcontrastoSeeTCEL-4stologiesPTCL-NOSativelativel•EATL•MEITLgodalPTCLTFHCLClinicaltrial(preferred)MultiagentMultiagentchemotherapym6cycles±ISRTnnthththcontrastoELgMEITLhasonlyrecentlybeenseparatedasitsownentityandoptimaltreatmenthasnotbeendefined.hPatientswithT-celllymphomasoftenhaveextranodaldisease,whichmaybeinadequatelyimagedbyCT.PETscanispreferred.kForselectedpatients,palliativetherapyforsymptommanagementmaybeconsidered.SeeTCEL-B2of7forpalliativetreatmentoptions.lALCL,ALK-negativewithaDUSP22rearrangementhasbeenvariablyassociatedwithaprognosismoresimilartoALK-positivediseaseandtreatmentaccordingtotheALCL,ALK-positivealgorithmmaybeconsideredforALK-negativeALCLwithDUSP22rearrangement(ParrillaCastellarER,etal.Blood2014;124:1473-1480;PedersenMB,etal.Blood2017;130:554-557;HapgoodG,etal.BrJHaematol2019;186:e28-e31).mSeeSuggestedTreatmentRegimens(TCEL-B).nSeePrinciplesofRadiationTherapy(TCLYM-D).oOtherbaselineimagingstudiesrelevantforresponseassessmentshouldberepeatedaswell.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version2.2022,03/07/22©2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.TCEL-3PrintedbyMinTangon3/14/20226:59:41AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dexDDITIONALTHERAPYDDITIONALAPYDDITIONALAPYRESPONSEClinical•H&Pforevery3–6moighdosetherapyHDTwithighdosetherapyHDTwithtologousstemcellrescueASCR)forhighriskIPIpatientsrCRp(PET••Surveillanceimagingh(nomoreoftenthanevery6mofor2yandthenannuallyforyorasclinicallyindicated)nedcourseoftmentmentPartialPPCTwithpositive)SeeRelapsed/RefractoryDisease(TCEL-6)responseorPrrtumorlysissyndromeSeeTCLYMBhPatientswithT