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NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)SystemicLightChainAmyloidosisersionJuneVersion1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.esPanelDisclosuresPrintedbyMinTangon9/2/202110:20:33AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllesPanelDisclosuresdosisdex*ShajiK.Kumar,MD/Chair‡ξMayoClinicCancerCenter*NatalieS.Callander,MD/ViceChair‡ξUniversityofWisconsineCancerCenterKehindeAdekola,MD,MSCI‡†RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityLarryAnderson,Jr.,MD,PhD‡†UTSouthwesternSimmonsComprehensiveCancerCenterMuhamedBaljevic,MD†‡ÞξFred&PamelaBuffettCancerCenterEricaCampagnaro,MD‡UniversityofMichiganRogelCancerCenterJorgeJ.Castillo,MD‡Dana-Farber/BrighamandWomen’sCancerCenter|MassachusettsGeneralHospitalCancerCenterCaitlinCostello,MD†‡ξUCSanDiegoMooresCancerCenterSrinivasDevarakonda,MD‡†TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteNouraElsedawy,MD†St.JudeChildren'sResearchHospital/TheUniversityofTennesseeeCenteratthewFaimanMDMBArehensiveCancerCenteriversityHospitalsSeidmanCancerCenternstituteAlfredGarfall,MD‡AbramsonCancerCenterheUniversityofPennsylvaniaKellyGodby,MD†O'NealComprehensiveCancerCenteratUABJensHillengass,MD,PhD‡RoswellParkComprehensiveCancerCenterLeonaHolmberg,MD,PhDξ‡FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceMyoHtut,MD‡ÞCityofHopeNationalMedicalCenterCarolAnnHuff,MD†‡TheSidneyKimmelComprehensiveCancerCenteratJohnsHopkinsMalinHultcrantz,MD,PhD‡†MemorialSloanKetteringCancerCenterYubinKang,MD‡†ξDukeCancerInstituteSarahLarson,MD†UCLAJonssonComprehensiveCancerCenterMichaelaLiedtke,MD‡StanfordCancerInstituteThomasMartin,MD‡UCSFHelenDillerFamilyComprehensiveCancerCenterJamesOmelMD¥eDouglasSborov,MD,MSc†‡ÞξHuntsmanCancerInstituteattheUniversityofUtahKennethShain,MD,PhD†MoffittCancerCenterKeithStockerl-Goldstein,MD†ξSitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineDonnaWeber,MD†‡ÞTheUniversityofTexasdiMScξBonemarrowtransplantation†Medicaloncology‡Hematology¥PatientadvocacyeeÞInternalmedicine*DiscussionsectioneeVersion1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.osisPanelMembersfGuidelinesUpdatesYLimaryTreatmentAMYLsisTherapyosisPanelMembersfGuidelinesUpdatesYLimaryTreatmentAMYLsisTherapyAMYLAntBasedonAmyloidosisConsensusCriteriaAMYLBghtChainAmyloidosisAMYLCfOrganandHematologicResponseandProgressionCriteriaAMYLDdosisdexFindanNCCNMemberInstitution:/home/member-institutions.dNCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.TheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2021.Version1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.vedtecrevisedIfNTproBNPisnotavailableBNPcanbeReferenceaddedforbendamustinedexamethasonezschSLagosvedtecrevisedIfNTproBNPisnotavailableBNPcanbeReferenceaddedforbendamustinedexamethasonezschSLagosGGComenzoRLetalBendamustinewithdexamethasoneinrelapsed/refractorysystemiclight-chainamyloidosis:ResultsofaphaseIIstudy.JClinOncol2020;38:1455-1462.Referenceaddedforcarfilzomib/dexamethasone:ManwaniR,etaneffectiveupfronttreatmentinALamyloidosispatientswithperipheralandautonomicneuropathy.BrJHaematol2019;187(5):638-641.sedrefractoryALamyloidosisBloodCancerJperformedIftroponinTisnotavailable,thentroponinIisacceptable.SpecialtestingbasedonorgansysteminvolvementVJetal.VenetoclaxinducesdeephematologicremissionsinpydosisdexsionoftheNCCNGuidelinesforSystemicLightomVersionincludeAMYL-1•Clinicalandamyloid-Footnoteerevised:Identificationoflightchainsintheserumorurinewithoutconfirmationoftheamyloidcompositionintissueisnotadequateaspatientswithotherformsofamyloidosismayhaveunrelatedmonoclonalgammopathyofundeterminedsignificance(MGUS).AMYL-2HCTcanelecttocollectstemcellsanddelaytransplanttoHCTcanelecttocollectstemcellsanddelaytransplanttoalaterlineAMYL-A1of5•Anewpagewasaddedtotheguidelines:GeneralConsiderationsforSystemicTherapyforSLCAAMYL-A2of5•PrimaryTherapyforHematopoieticCellTransplantEligibleCandidatesandNon-EligibleCandidates:pThefollowingwasmovedfromPreferredtoOtherRecommended:◊Bortezomib/cyclophosphamide/dexamethasonepThefollowingwereremovedfromOtherRecommended:◊Lenalidomide/cyclophosphamide/dexamethasone◊Lenalidomide/dexamethasone•Footnotesweremovedtoanewpage:GeneralConsiderationsforSystemicTherapyforSLCA(AMYL-A1of5).•Footnoteaadded:SeeGeneralConsiderationsforSystemicTherapyforSLCA(AMYL-A1of5).AMYL-A3of5AMYL-A3of5dosisdexamethasonedosisdexamethasoneFootnotesweremovedtoanewpage:GeneralConsiderationsforSystemicTherapyforSLCAAMYLAof5).FootnoteaaddedSeeGeneralConsiderationsforSystemicTherapyforSLCAAMYLA1of5).AMYL-A4of5•Referenceaddedforixazomib/lenalidomide/dexamethasone:CohenOC,SharpleyF,GilmoreJD,etal.Useofixazomib,lenalidomideanddexamethasoneinpatientswithrelapsedReferenceaddedforvenetoclax/dexamethasone:Premkumaramyloidlight-chainReferenceaddedforvenetoclax/dexamethasone:PremkumarPeripheralbloodsmear•Prothrombintime(PT),partialthromboplastintime(PTT),andFactorX(ifSerumBUNcreatinineelectrolytes,albumin,calcium,serumuricacid,serummicroglobulinCreatinineclearancecalculatedormeasureddirectly)Serumquantitativeimmunoglobulins,serumproteinelectrophoresis(SPEP),andserumimmunofixationelectrophoresisSIFE)Peripheralbloodsmear•Prothrombintime(PT),partialthromboplastintime(PTT),andFactorX(ifSerumBUNcreatinineelectrolytes,albumin,calcium,serumuricacid,serummicroglobulinCreatinineclearancecalculatedormeasureddirectly)Serumquantitativeimmunoglobulins,serumproteinelectrophoresis(SPEP),andserumimmunofixationelectrophoresisSIFE)4-hurinefortotalprotein,urineproteinelectrophoresis(UPEP),andurineionelectrophoresisUIFESerumfreelightchain(FLC)assayAlkalinephosphataseaspartateaminotransferase(AST),alanineALTandbilirubinCoagulationstudiesasindicatedrrow•Abdominalfatpadsamplinggand/orinvolvedorganbiopsyasclinicallyindicatedAmyloidtissuesubtypingwithmassspectrometryinverandGItractftodocumentcraniocaudalsclinicallyindicatedripheralnervoussystemsignificantperipheralneuropathyonstudiescortisolunctiontestsCThasbeenperformed,thenskeletalsurveyisnotneeded.cIfNTproBNPisnotavailableBNPcanbeperformed.IftroponinTisnotavailable,thentroponinIisacceptable.paresispresentherdItisessentialtoconfirmthatpatientshaveprimarysystemiclightchainamyloidosisALratherthanhereditaryamyloidosis,wild-typetransthyretin-related(ATTR)cardiacamyloidosis,dosisdexINITIALDIAGNOSTICWORKUPaClinicalandamyloid-relatedassessment•Historyandphysical(H&P)•Whole-bodylow-dose•Whole-bodylow-doseCTscanbECGifferentialplateletcountluationdirectedifferentialplateletcountUnilateralbonemarrowaspirateUnilateralbonemarrowaspirate+biopsyfinvolvement•Cardiaccialtestingbasedoninvolvement•CardiacsmenttoexaminesmenttoexaminelongitudinalbSkeletalsurveyisacceptableincertaincircumstancesHoweveritissignificantlylesssensitivethanwholebodylowbSkeletalsurveyisacceptableincertaincircumstancesHoweveritissignificantlylesssensitivethanwholebodylowdoseCTandFDGPETCTIfFDGPETCTorwholebodylowdoseorsecondaryamyloidosis.Theamyloiddepositsshouldbeconfirmedtobecomposedoflightchainsusingimmunohistochemistryormassspectrometry.ImmunohistochemistryfortransthyretinorserumamyloidAcomponentshouldbeperformedifkappaandlambdastainsarenegative.Tc-pyrophosphatescancanhelpdistinguishcardiacinvolvementwithALeIdentificationofeIdentificationoflightchainsintheserumorurinewithoutconfirmationoftheamyloidcompositionintissueisnotadequate,aspatientswithotherformsofamyloidosismayhaveanunrelatedmonoclonalgammopathyofundeterminedsignificance(MGUS).LachmannHJ,etal.Nunrelatedmonoclonalgammopathyofundeterminedsignificance(MGUS).LachmannHJ,etal.NEnglJMed2002;346:1786-1791.fCongoredstainingforamyloid.Congostaindoesnotdifferentiatebetweentypesofamyloid.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.AMYL-1Version1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon9/2/202110:20:33AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dosisdexCLINICALFINDINGSTREATMENTOFNEWLYDIAGNOSEDDISEASEa,h,iRELAPSED/REFRACTORYDISEASEiOrganinvolvementbasedonamyloidosiscriteriahcriteriahEvaluateforstemcelljltransplantjlClinicaltrialTherapyTherapyfornewlydiagnoseddiseasek(seeAMYL-A)andBestsupportivecareSeeNCCNGuidelinesforPalliativeCareSeeNCCNGuidelinesforSurvivorshipTherapyforrelapsed/refractorydisease(SeeAMYL-A)andBestsupportivecareSeeNCCNGuidelinesforPalliativeCareaFrailtyassessmentshouldbeconsideredinolderadults.SeeNCCNGuidelinesforOlderAdultOncology.hSeeDefinitionofOrganInvolvementandResponsetoTreatmentBasedonAmyloidosisConsensusCriteria(AMYL-B).iSeeDefinitionofOrganandHematologicResponseandProgressionCriteria(AMYL-C).jInthosepatientswithverylowtumorburdeninductiontherapymaynotberequired.IfnotacandidateforSCTatinitialdiagnosis,reassessafter2cyclesofsystemictherapy.kOrgantransplant,asclinicallyindicated.lPatientseligibleforhematopoieticcelltransplantcanelecttocollectstemcellsanddelaytransplanttoalaterlineoftherapy.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.AMYL-2PrintedbyMinTangon9/2/202110:20:33AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dosisdexTEMICTHERAPYFORSLCAGeneralPrinciples•Frailtyassessmentshouldbeconsideredinolderadults.SeeNCCNGuidelinesforOlderAdultOncology.•Consideroraldoxycyclineasadjuvanttostandardsystemictherapy.ScreeningRecommendations•ScreenforHIVandhepatitisC,asclinicallyindicated.•TestforhepatitisBbeforestartingcarfilzomibordaratumumab.•Daratumumabmayinterferewithserologictestingandcausefalse-positiveindirectCoombstest.Typeandscreenshouldbeperformedbeforeusingdaratumumab.ProphylaxisRecommendations•Recommendherpeszosterprophylaxisforpatientstreatedwithproteasomeinhibitorsordaratumumab.SideEffectsandLabTests•Regimenscontainingbortezomibareassociatedwithhigherriskoftreatment-relatedperipheralneuropathy,especiallyinthosewithdisease-relatedbaselineneuropathy.Closemonitoringoralternativetherapiesshouldbeconsideredinsomepatients.•Carfilzomibcanpotentiallycausecardiacandpulmonarytoxicity,especiallyinelderlypatients.•Patientswithcardiacamyloidshouldbecarefullymonitoredwhileonlenalidomidetherapy.DosingandAdministration•Thedoseofmelphalanaspartofhematopoieticcelltransplantationcanbeadjustedbasedonfactorssuchasage,presence/absenceofcardiacinvolvement,andnumberoforgansinvolved.Theserisk-adaptedapproacheshavenotbeenevaluatedinrandomizedstudies.•Proteasomeinhibitors:pSubcutaneousbortezomibisthepreferredmethodofadministration.pBothweeklyandtwice-weeklydosingschemasofbortezomibmaybeappropriate;weeklypreferred.pCarfilzomibmaybeusedonceortwiceweeklyandatdifferentdoses.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.AMYL-AOF5PrintedbyMinTangon9/2/202110:20:33AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dosisdexPRIMARYTHERAPYFORHEMATOPOIETICCELLTRANSPLANTELIGIBLECANDIDATESANDNON-ELIGIBLECANDIDATESa(Orderofregimensisalphabeticalanddoesnotindicatepreference)(Note:ifnotacandidateforhematopoieticcelltransplantatinitialdiagnosis,reassessafter2cyclesofsystemictherapy)PreferredRegimen:•Daratumumabandhyaluronidase-fihj/bortezomib/cyclophosphamide/dexamethasone(category1)OtherRecommendedRegimens:•Bortezomib±dexamethasone•Bortezomib/cyclophosphamide/dexamethasonetezomiblenalidomidedexamethasonetezomibmelphalandexamethasonehalandexamethasoneaSeeGeneralConsiderationsforSystemicTherapyforSLCAAMYLAof5).Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.AMYL-A2OF5AMYL-A3OF5PrintedbyMinTangon9/2/202110:20:33AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllAMYL-A3OF5dosisdexTHERAPYFORPREVIOUSLYTREATEDDISEASEaConsiderrepeatinginitialtherapy,especiallyifrelapse-freeforseveralyears•High-dosemelphalanwithhematopoieticcelltransplant•Bortezomib±dexamethasone•Daratumumabb•Bortezomib/•Daratumumabb•Ixazomib±dexamethasone•Ixazomib/lenalidomide/dexamethasonealidomidecyclophosphamidedexamethasonelidomidedexamethasonehalandexamethasonelidomidedexamethasoneUsefulinCertainCircumstances•Bendamustine/dexamethasone•Carfilzomibfornon-cardiacamyloidosis±dexamethasone•Venetoclaxt(11;14)±dexamethasoneaSeeGeneralConsiderationsforSystemicTherapyforSLCA(AMYL-A1of5).bIncludesbothdaratumumabforintravenousinfusionanddaratumumabandhyaluronidase-fihjforsubcutaneousinjection.Daratumumabandhyaluronidase-fihjforsubcutaneousinjectionhasdifferentdosingandadministrationinstructionscomparedtodaratumumabforintravenousinfusion.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.idosisresultsofaphasestudyBloodPrintedbyMinTangon9/2/202110:20:33AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalidosisresultsofaphasestudyBlooddosisdexSYSTEMICLIGHTCHAINAMYLOIDOSISTHERAPYREFERENCESFORTREATMENTOPTIONS•Bendamustine/dexamethasonepLentzschS,LagosGG,ComenzoRL,etal.Bendamustinewithdexamethasoneinrelapsed/refractorysystemiclight-chainamyloidosis:ResultsofaphaseIIstudy.JClinOncol2020;38:1455-1462.•Bortezomib/cyclophosphamide/dexamethasonepVennerCP,LaneT,FoardD,etal.Cyclophosphamide,bortezomib,anddexamethasonetherapyinALamyloidosisisassociatedwithhighclonalresponseratesandprolongedprogression-freesurvival.Blood2012;119:4387-4390.pMikhaelJR,SchusterSR,Jimenez-ZepedaVH,etal.Cyclophosphamide-bortezomib-dexamethasone(CyBorD)producesrapidandcompletehematologicresponseinpatientswithALamyloidosis.Blood2012;119:4391-4394.zomibinpatientswithrelapsedsystemicpKastritisE,WechalekarAD,DimopoulosMA,etal.Bortezomibwithorwithoutdexamethasoneinprimarysystemic(lightchain)amyloidosis.JClinOncol2010;28:1031-1037.pSinghV,SaadA,PalmerJ,etal.Responsetobortezomibbasedinductiontherapyinnewlydiagnosedlightchain(AL)amyloidosis[abstract].Blood2009;114:Abstract1867.pLammW,WillenbacherW,LangA,etal.EfficacyofthecombinationofbortezomibanddexamethasoneinsystemicALamyloidosis.AnnHematol2011;90:201-206.pReeceDE,SanchorawalaV,HegenbartU,etal.Weeklyandtwice-weeklybortezomibinpatientswithsystemicALamyloidosis:resultsofaphase1dose-escalationstudy.Blood2009;114:1489-1497.•Bortezomib/lenalidomide/dexamethasonepKastritisE,DialoupiI,GavriatopoulouM,etal.Primarytreatmentoflight-chainamyloidosiswithbortezomib,lenalidomide,anddexamethasone.BloodAdv2019;3:3002-3009.•Bortezomib/melphalan/dexamethasonepGasparettoC,SanchorawalaV,SnyderRM,etal.Useofmelphalan(M)/dexamethasone(D)/bortezomibinALamyloidosis[abstract].JClinOncol2010;28:Abstract8024.•Carfilzomib/dexamethasonepManwaniR,MahmoodS,SachchithananthamS,etal.CarfilzomibisaneffectiveupfronttreatmentinALamyloidosispatientswithperipheralandautonomicneuropathy.BrJHaematol2019;187:638-641.•DaratumumabpKaufmanGP,SchrierSL,LafayetteRA,etal.DaratumumabyieldsrapidanddeephematologicresponsesinpatientswithheavilypretreatedALamyloidosis.Blood2017;130:900-902.newlydiagnosedALamyloidosissafetyruninresultsofANDROMEDA.Blood2020;136:71-80.aVSeldinDaVSeldinDetalHighdosemelphalanandautologousstemcelltransplantationinpatientswithALamyloidosisanyearstudy.AnnInternMed2004;140:85-93.pGertzMA,LacyMQ,DispenzieriA,etal.Risk-adjustedmanipulationofmelphalandosebeforestemcelltransplantationinpatientswithamyloidosisisNote:AllrecommendationsNote:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.AMYL-AVersion1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.4OF5PrintedbyMinTangon9/2/202110:20:33AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2021NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.dosisdexSYSTEMICLIGHTCHAINAMYLOIDOSISTHERAPYREFERENCESFORTREATMENTOPTIONSassociatedwithalowerresponserate.BoneMarrowTransplant2004;34:1025-1031.pPerfettiV,SienaS,PalladiniG,etal.Long-termresultsofarisk-adaptedapproachtomelphalanconditioninginautologousperipheralbloodstemcelltransplantationforprimary(AL)amyloidosis.Haematologica2006;91:1635-1643.pD’SouzaA,DispenzieriA,WirkB,etal.Improvedoutcomesafterautologoushematopoieticcelltransplantationforlightchainamyloidosis:Acenterforinternationalbloodandmarrowtransplantresearchstudy.JClinOncol2015;33:3741-3749.pDispenzieriA,SeenithambyK,LacyMQ,etal.Patientswithimmunoglobulinlightchainamyloidosisundergoingautologousstemcelltransplantationhavesuperioroutcomescomparedwithpatientswithmultiplemyeloma:aretrospectivereviewfromatertiaryreferralcenter.BoneMarrowTransplant2013;48:1302-1307.sBlood2017;130:597-605.•Ixazomib/lenalidomide/dexamethasonepCohenOC,SharpleyF,GilmoreJD,etal.Useofixazomib,lenalidomideanddexamethasoneinpatientswithrelapsedamyloidlight-chainamyloidosis.BrJHaematol2020;189:643-649.•Lenalidomide/cyclophosphamide/dexamethasonepKumarSK,HaymanSR,BuadiFK,etal.Lenalidomide,cyclophosphamide,anddexamethasone(CRd)forlight-chainamyloidosis:long-termresultsfromaphase2trial.Blood2012;119:4860-4867.pPalladiniG,RussoP,MilaniP,etal.AphaseIItrialofcyclophosphamide,lenalidomideanddexamethasoneinpreviouslytreatedpatientswithALamyloidosis.Haematologica2013;98:433-436.•Lenalidomide/dexamethasonepSanchorawalaV,WrightD,RosenzweigM,etal.LenalidomideanddexamethasoneinthetreatmentofALamyloidosis:resultsofaphase2trial.Blood2007;109:492-496.pDispenzieriA,LacyM,ZeldenrustS,etal.Theactivityoflenalidomidewithorwithoutdexamethasoneinpatientswithprimarysystemicamyloidosis.Blood2007;109:465-470.pDispenzieriA,LacyM,ZeldenrustS,etal.Longtermfollow-upofpatientswithimmunoglobulinlightchainamyloidosistreatedwithlenalidomideanddexamethasone[abstract]Blood2008;112:Abstract1737.lood2007;110:787-788.pJaccardA,LeblondV,RoyerB,etal.Autologousstemcelltransplantation(ASCT)versusoralmelphalanandhigh-dosedexamethasoneinpatientswithAL(primary)amyloidosis:longtermfollow-upoftheFrenchmulticentricrandomizedtrial[abstract].Blood2010;116:Abstract1344.•Pomalidomide/dexamethasonepDispenzieriA,BuadiF,LaumannK,etal.Activityofpomalidomideinpatientswithimmunoglobulinlight-chainamyloidosis.Blood2012;119:5397-5404.pSanchorawalaV,SheltonA,LoS,etal.PomalidomideanddexamethasoneinthetreatmentofALamyloidosis:Resultsofaphase1and2trial.Blood2016;128:1059-1062.myloidosisBloodCancerJ1;11:10.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.Version1.2022,06/29/2021©2021NationalComprehensiveCancerNetwork(NCCN),Allrigh

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