T細胞淋巴瘤2022.v1（英文）-NCCN腫瘤臨床實踐指南

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?TMemorialSloanKetteringCancerCenter*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,MD?TheUniversityofTexasAhmetDogan,MD,PhD≠MemorialSloanKetteringCancerCenterFrancineFoss,MD??ξYaleCancerCenter/SmilowCancerHospital*PaolaGhione,MD?RoswellParkComprehensiveCancerCenter*AaronM.Goodman,MD?ξUCSanDiegoMooresCancerCenterJoanGuitart,MD≠?RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityesPanelDisclosures*AhmadHalwani,MD?HuntsmanCancerInstitute*AhmadHalwani,MD?HuntsmanCancerInstituteattheUniversityofUtah*BradleyM.Haverkos,MD,MPH,MS?UniversityofWisconsinCarboneCancerCenterSimaRozati,MD,PhD?ofColoradoCancerCenterRichardT.Hoppe,MDofColoradoCancerCenterRichardT.Hoppe,MD§StanfordCancerInstituteJacobsenMD*JonathanSaid,MD≠UCLAJonssonComprehensiveCancerCentermandWomensentermandWomensenter*DeepaJagadeesh,MD,MPH??CaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussignstituteAllisonJones,MD?St.JudeChildren'sResearchHospital/TheUniversityofTennesseeHealthScienceCenterAvyaktaKallam,MD,MBBSTVanderbilt-IngramCancerCenter*LaurenShea,MD?O'NealComprehensiveCancerCenteratUAB*MichiM.Shinohara,MD?≠FredHutchinsonCancerResearchCenter/SeattleCancerCareAlliance*LubomirSokol,MD,PhD??TMoffittCancerCenterCarlosTorres-Cabala,MD≠TheUniversityofTexasfettfettCancerCenterYounH.Kim,MD??StanfordCancerInstituteKiranKumar,MD,MBA§UTSouthwesternSimmonsComprehensiveCancerCenter*NehaMehta-Shah,MD,MSCI??*RyanWilcox,MD,PhD??UniversityofMichiganRogelCancerCenter*PeggyWu,MD,MPH?UCDavisComprehensiveCancerCenterJasmineZain,MD??nCancerCenteratBarnesCitynCancerCenteratBarnesalandWashingtonoolofMedicineEliseA.Olsen,MD??DukeCancerInstituteξBonemarrowtransplantation≠Pathology?Dermatology?Plasticsurgery?Hematology/Hematology§Radiotherapy/RadiationoncologyoncologyTInternalmedicine*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,TCRα??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,TCR?,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