急性髓细胞白血病2022.v1（英文）-NCCN肿瘤临床实践指南

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines®)AcuteMyeloidLeukemiarsionDecemberNCCNGuidelinesforPatients®availableat/patientsVersion1.2022,12/02/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:26:15AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022AcuteMyeloidLeukemia(Age≥18years)dex*DanielA.Pollyea,MD,MS/Chair‡Þ†UniversityofColoradoCancerCenterJessicaK.Altman,MD/ViceChair‡RobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityVijayaRajBhatt,MBBS‡ξFred&PamelaBuffettCancerCenterDaleBixby,MD,PhD‡†ÞUniversityofMichiganRogelCancerCenterHettyCarraway,MD,MBA†CaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussigCancerInstituteAmirT.Fathi,MD‡†MassachusettsGeneralHospitalCancerCenterJamesM.Foran,MD†MayoClinicCancerCenterIvanaGojo,MD‡TheSidneyKimmelComprehensiveCancerCenteratJohnsHopkinsAricC.Hall,MD‡†ξUniversityofWisconsineCancerCenterMeaganJacoby,MD,PhD‡†ξSitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineBrianA.Jonas,MD,PhD‡UCDavisComprehensiveCancerCenteresPanelDisclosuresJeffreyLancet,MD‡†MoffittCancerCenterJamesMangan,MD‡UCSanDiegoMooresCancerCenterGabrielMannis,MD‡ÞStanfordCancerInstituteGuidoMarcucci,MD†ÞCityofHopeNationalMedicalCenterAliceMims,MD,MS†TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteJadeeNeff,MD,PhD≠DukeCancerInstituteRezaNejati,MD≠FoxChaseCancerCenterRebeccaOlin,MD,MS‡ξUCSFHelenDillerFamilyComprehensiveCancerCenterPraptiPatel,MD‡UTSouthwesternSimmonsComprehensiveCancerCenterMary-ElizabethPercival,MD,MS‡FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceAlexanderPerl,MD†AbramsonCancerCenterattheUniversityofPennsylvaniaAmandaPrzespolewski,DO‡RoswellParkComprehensiveCancerCenterDineshRao,MD,PhD≠UCLAJonssonComprehensiveCancerCenterFarhadRavandi,MDÞ‡TheUniversityofTexasPaulJ.Shami,MD‡HuntsmanCancerInstituteattheUniversityofUtahRichardM.Stone,MD‡†Dana-Farber/BrighamandWomen’senterStephenA.Strickland,MD,MSCI‡Vanderbilt-IngramCancerCenterKendraSweet,MD,MS‡Þ†MoffittCancerCenterMartinTallman,MD‡MemorialSloanKetteringCancerCenterSwapnaThota,MD‡St.JudeChildren'sResearchHospital/TheUniversityofTennesseeHealthScienceCenterPankitVachhani,MD‡O'NeilComprehensiveCancerCenteratUABCampbellPhD‡ÞξBonemarrow‡Þ*Hematology/*InternalmedicineMedicaloncologyPathologyDiscussionSectionWritingCommitteeMemberVersion1.2022,12/02/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.eatmentofCNSLeukemia•PrinciplesofRadiationTherapy(AML-C)•GeneralConsiderationsandSupportiveCareforAMLPatientsWhoPreferNottoeatmentofCNSLeukemia•PrinciplesofRadiationTherapy(AML-C)•GeneralConsiderationsandSupportiveCareforAMLPatientsWhoPreferNottoReceiveBloodTransfusions(AML-D)•PrinciplesofSupportiveCareforAML(AML-E)•MonitoringDuringTherapy(AML-F)•Measurable(Minimal)ResidualDiseaseAssessment(AML-G)•ResponseCriteriaDefinitionsforAcuteMyeloidLeukemia(AML-H)•TherapyforRelapsed/RefractoryDisease(AML-I)•PrinciplesofVenetoclaxUsewithHMAorLDAC(AML-J)•Introduction(BPDCN-INTRO)•Evaluation/Workup(BPDCN-1)•Treatment(BPDCN-2)•SurveillanceandTreatmentforRelapsed/RefractoryDisease(BPDCN-3)•PrinciplesofBPDCN(BPDCN-A)•EvaluationandTreatmentofCNSDisease(BPDCN-B)•PrinciplesofSupportiveCareforBPDCN(BPDCN-C)NCCNGuidelinesVersion1.2022AcuteMyeloidLeukemia(Age≥18years)dexlievesthatthebestmanagementlievesthatthebestmanagementforanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.FindanNCCNMemberInstitution:/home/member-institutions.ofEvidenceandsusAllrecommendationsotherwisedNCategoriesofEvidenceandConsensus.NCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.SummaryofGuidelinesUpdatesMLeticsMLeticsinNonAPLAMLPL•ClassificationandTreatmentRecommendations(APL-1)•Low-RiskTreatmentInductionandConsolidationTherapy(APL-2)•High-RiskInductionandConsolidationTherapy(APL-3)•Post-ConsolidationTherapyandMonitoring(APL-5)•TherapyforRelapse(APL-6)•PrinciplesofSupportiveCare(APL-A)ML•(Age<60y)TreatmentStrategiesandInduction(AML-1)•(Age<60y)RiskStatusandConsolidationTherapy(AML-4)•(Age≥60y)TreatmentStrategiesandInduction-CandidatesforIntensiveTherapy(AML-5)•(Age≥60y)TreatmentStrategiesandInduction-Non-CandidatesforIntensiveTherapy(AML-6)•(Age≥60y)AfterStandard-DoseCytarabineInduction(AML-7)•(Age≥60y)Post-InductionTherapy-PreviousIntensiveTherapy(AML-8)•(Age≥60y)Post-InductionTherapy-PreviousLowerIntensityTherapy(AML-9)•MaintenanceTherapy(AML-10)•AMLSurveillanceandTherapyforRelapsed/RefractoryDisease(AML-11)TheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatment.AnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualclinicaltancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanyway.TheNCCNbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.©2021.Version1.2022,12/02/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:26:15AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022AcuteMyeloidLeukemia(Age≥18years)dexheNCCNGuidelinesforAcuteMyeloidLeukemiafromVersionincludeuationp3rdbulletadded:B12andfolicacidevaluationp14thbulletadded:Considerearlyintegrationofpalliativecare(SeeNCCNGuidelinesforPalliativeCare)•DiagnosispAPLrevised:InpatientswithclinicalandorpathologicfeaturesofAPLpAML,1stsub-bulletrevised:...cytarabine(1-2g)maybeconsideredpriortoreceivingdiagnosticresultsEVAL-1A•Footnotearevised:...decision-making(category2B)(SeeAML-A).Othergeneticlesions,suchasASXL1,BCR-ABL,andPML-RARalpha(SeeAML-A)mayhavetherapeuticimplicationssignificance...WhiletheaboveMutationsshouldbetestedinallpatients...•Footnotecadded:El-JawahriA,etal.JAMAOncol2021;7:238-245.AcutePromyelocyticLeukemia(Age≥18years)APL-2•APL(LowRisk)pTreatmentinduction,PreferredRegimen,1stand2ndpathwaysrevised:ATRA45mg/m2in2divideddosesdaily+arsenictrioxide...pConsolidationtherapy,2ndrowrevised:First3consolidationcycles=56daycycles;ATRA45mg/m2/dPOdividedintoin2divideddailydoseson...pTreatmentinduction,UsefulinCertainCircumstances,3rdpathwayrevised:ATRA45mg/m2in2divideddosesdaily+idarubicin...pConsolidationtherapy,4thpathwayrevised:ATRA45mg/m2in2divideddosesdailymaybegivenmonthlyuntil28weeksfromcompleteresponse(CR)untilachievementofcompletemolecularresponse.APL-3•APL(HighRisk),Treatmentinduction,ATRAregimenwasclarifiedasappropriate:ATRA45mg/m2in2divideddosesdaily...APL-3A•Footnotepadded:ItisimportantforthemanagementofAPLthatregimenscontainingATRAandarsenictrioxidebeadministeredunlessthereisacontraindicationbasedonareextenuatingpatientcircumstances.ItisimportantforregimenscontainingATRAandarsenictrioxidetobeadministeredforthemanagementofAPL.Ifarsenicisnotavailableorcontraindicated,itmaybeomittedfrominduction.•Footnotesadded:Noarsenicisincludedininductionifunavailableorcontraindicated.•Footnotexrevised:Consider4–6dosesofITchemotherapy(eg,2dosesforeachconsolidationcycle)asanoptionforCNSprophylaxis.APL-4•APL(HighRisk)inPatientswithCardiacIssuespTreatmentinduction,ATRAregimenwasclarifiedasappropriate:ATRA45mg/m2in2divideddosesdailypConsolidationTherapy,ProlongedQTc◊1stpathway:ATRA45mg/m2in2divideddosesuntil28weeksfromCRuntilachievementofcompletemolecularresponse.◊3rdpathway:...+cytarabine150mg/m2/8hover8hx4daysx1cycle.APL-4A•Footnoteremoved:ForpatientswhohaveprolongedQTcastheirsolecomorbidity,gemtuzumabozogamicincouldbesubstitutedforanthracycline.UPDATESVersion1.2022,12/02/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:26:15AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022AcuteMyeloidLeukemia(Age≥18years)dexheNCCNGuidelinesforAcuteMyeloidLeukemiafromVersionincludeAcutePromyelocyticLeukemia(Age≥18years)(continued)APL-5•Footnotebbadded:GrimwadeD,etal.JClinOncol2009;27:3650-3658.APL-6•Footnoteddadded:CicconiL.etal.AnnHematol2018;97:1797-1802.APL-A•PrinciplesofSupportiveCareforAPLp3rdbullet,2ndsub-bulletrevised:dexamethasone10mgq12h(SeeNCCNPreventionandTreatmentofCancer-RelatedInfections).pFootnote1added:Antiviralprophylaxiszosterfordurationoftreatmentmaybeappropriate.FreyerCW,etal.LeukLymphoma2021;62:696-702;GlassJL,etal.Blood2015;126:3752–3752.pFootnote2added:DaverN,etal.BrJHaematol2015;168:646-53.AcuteMyeloidLeukemia(Age≥18years)AML-1•General:PhysiologicwasremovedfromagethroughoutAMLsection•Headerclarifiedas:Age<60y,Inductioneligible•TreatmentStrategiesp2ndqualifierrevised:Intermediate-riskcytogeneticsandFLT3-mutated(ITDorTKD)toFLT3/ITD/TKDwithintermediatepoor-riskgeneticsAML-1A•Footnotecrevised:PatientswithCBF-AMLandcoreabnormalitiesmaybenefitfromtheadditionofgemtuzumabozogamicinGemtuzumabozogamicinisnotbeneficialinpatientswithadverseriskAML.•Footnotedadded:BorlenghiE,etal.JGeriatrOncol2021;12:550-556.•Footnoteeadded:ForCBF-AMLleukemiawithFLT3mutation,thepanelprefersgemtuzumabozogamicin.•Footnotefadded:GemtuzumabozogamicinmaybebeneficialinNPM1-mutatedAML(Kapp-SchwoererS,etal.Blood2020;136:3041-3050).•Footnotepadded:AnFDA-approvedbiosimilarisanappropriatesubstituteforfilgrastim.•Footnotesadded:Outcomeswithunfavorable-riskcytogeneticsandTP53-mutatedAMLremainpoorwithconventionalinductionchemotherapy(RückerFG,etal.Blood2012;119:2114-2121).Considerclinicaltrials,azacitidine/venetoclax(DiNardoCD,etal.NEnglJMed2020;383:617-629),ora10-daycourseofdecitabine(WelchJS,etal.NEnglJMed2016;375:2023-2036).•Footnoteurevisedbyremoving:However,onestudyshowedthathigh-dosecytarabinemayimprovetheoutcomeforyoungerpatients.WillemzeR,etal.JClinOncol2014;32:219-228.AML-2-AGE<60yAFTERSTANDARD-DOSECYTARABINEINDUCTION/RE-INDUCTION•Significantcytoreductionwithlow%residualblasts,3rdbulletadded:Intermediateorhigh-dosecytarabineVersion1.2022,12/02/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESPOoncedailyondaysofeachdaycycleuntilprogressionorunacceptabletoxicityifpatientsPOoncedailyondaysofeachdaycycleuntilprogressionorunacceptabletoxicityifpatientstenancedeclineorarenotfiteligibleforallogeneicHCT)NCCNGuidelinesVersion1.2022AcuteMyeloidLeukemia(Age≥18years)dexheNCCNGuidelinesforAcuteMyeloidLeukemiafromVersionincludeAML-4eNPMpositiveFLTnegativeAML-5FootnoteremovedfrompageInpatientswithAMLwithTP53mutation,a10-daycourseofdecitabinemaybeconsidered(WelchFootnoteremovedfrompageInpatientswithAMLwithTP53mutation,a10-daycourseofdecitabinemaybeconsidered(WelchJS,etal.NEnglJMed2016;375:2023-2036).ResponsemaynotbeevidentbeforeNEnglJMed2016;375:2023-2036).Responsemaynotbeevidentbefore3–4cyclesoftreatmentwithHMAs(ie,azacitidine,decitabine).reatmentuntilprogressionifpatientistoleratingtherapySimilardelaysinresponsearelikelywithnovelagentsinaclinicaltrial,butendpointswillbedefinedbytheprotocol.TMENTINDUCTION•IDH1orIDH2mutation,forbothpreferredandotherrecommended,thevenetoclax-basedregimenmovedtofirstbullet.•Footnoteeeeadded:DiNardoCD,etal.NEnglJMed2020;383:617-629.AML-8•Post-Remission/MaintenanceTherapyheaderrevisedasPost-InductionTherapy•Aftercompleteresponse,thepathwaywassplitby"Abletoreceiveconventionalconsolidation"and"Notabletoreceiveanyorallofrecommendedconsolidation•Maintenancetherapyoptionsmovedtonotabletoreceiveanyorallofrecommendedconsolidation.toxicitywaschangedfromacategory2Atoacategory1,preferredgressiondecitabinechangedfromacategoryAtoacategory2Brecommendation.ntendedtoreplaceconsolidationchemotherapywhichcanbecurativeinsomecasesInadditionfitpatientswithbecurativeinsomecasesInadditionfitpatientswithintermediateand/oradverse-riskcytogeneticsmaybenefitfromHCTinfirstCR,erearenodatatosuggestthatmaintenancetherapywithoralazacitidinecanreplaceHCTThepanelalsonotesthatthetrialdidnotincludeyoungerpatientsorthosewithwithCBFAMLitwasrestrictedtopatients≥55yearsofagewithintermediateoradverseticswhowerenotfelttobecandidatesforHCTMostpatientsreceivedatleastcycleofconsolidationpriortostartingoralazacitidineWeiAHetalBloodSupplLBAWeiAHetalNEnglJMed2020;383:2526-2537."FootnoteooowasrevisedAnoptionforpatientswhohadachievedaremissionwithamoreintensiveregimenbuthadregimen-relatedtoxicitythatpreventedthemfromreceivingmoreconventionalconsolidation.Azacitidine:HulsG,etal.Blood2019;133:1457-1464;DecitabineBoumberYetalLeukemia428–3241.AML-10MAINTENANCETHERAPYgeneicstemcelltransplantationinremissionandhistoryofFLTITDwiththeoptionofFLTinhibitormaintenancewithsorafenibasacategory2Arecommendation.Clarifiedcriteriafororalazacitidineashemotherapyandisnowinremissiononsomeconsolidationorarecommendedcourseofconsolidationandtisplanned•Added:Neitheroftheabovescenariosisapplicablewiththerecommendationformaintenancetherapyasnotrecommended.Version1.2022,12/02/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESUPDATESVersion1.2022,12/02/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon3/14/20227:26:15AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright©2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.NCCNGuidelinesVersion1.2022AcuteMyeloidLeukemia(Age≥18years)dexUpdatesinVersion1.2022oftheNCCNGuidelinesforAcuteMyeloidLeukemiafromVersion3.2021include:AML-A2of4FAMILIALGENETICALTERATIONSINAML•NewpageaddedrelatedtopredispositiontoAML.AML-H-RESPONSECRITERIADEFINITIONSFORACUTEMYELOIDLEUKEMIA•Statementadded:Theseresponsecriteriaweredefinedinthecontextofintensivechemotherapyregimens,andmaynotbepredictiveofoutcomesforpatientswhoreceiveothertherapies.AML-I-THERAPYFORRELAPSED/REFRACTORYDISEASE•Footnote8added:AnFDA-approvedbiosimilarisanappropriatesubstituteforfilgrastim.AML-J-PRINCIPLESOFVENETOCLAXUSEWITHHMAORLDAC•General,2ndbulletrevisedfrom,"ReductionindurationofHMAandLDACorvenetoclaxtreatmentcanbeconsidered,particularlywhentherearedelaysincountrecovery"to"Wheretherearedelaysincountrecovery,reductionindurationofvenetoclaxand/orreductionindoseordurationofHMAorLDACshouldbeconsidered."•TherapyforNewlyDiagnosedPatientspPriortotherapysub-bulletsrevised:◊Todecreasetheriskofseveretumorlysissyndrome(TLS),aimtrytoachieveWBCcountof<25,000/mcLwithhydroxyurea/leukapheresisifnecessary.◊AdministerInitiateboththerapiesofthecombinationconcomitantly.◊IfazoleantifungalprophylaxisorotherCYPenzymeinteractingmedicationsareconcurrentlyindicated,reducevenetoclaxdoseaccordingly.pFirstCycleConsiderations◊TLSmonitoring,2ndsub-bulletrevisedbyadding:Concomitantinteractingmedicationsmayrequirechangestothesedosages.◊TLSmonitoring,4thsub-bulletrevised:Forpatientswithproliferativedisease,monitorbloodchemistriesevery6–8hoursafterinitiation;ifwithinnormallimits,recheckoncedailyandcontinuemonitoringuntilnofurtherriskofTLS.◊3rdsub-bulletrevised:Ifnomorphologicremission(persistentmarrowblastsabove5%)...pCycle2andbeyond◊3rdsub-bulletadded:Ifpersistentdiseaseaftercycle1,repeatmarrowbiopsyfollowingcycle2(orsubsequentcyclesuntilNEDorremission)toagainassessforcellularityanddiseaseresponse,anddeterminetimingofsubsequentcycle.◊6thsub-bulletrevised:Ifnomorphologicremissionaftercycle2or3,thelikelihoodofresponseisdecreasedandpatientscouldconsidersmacytoidDendriticCellNeoplasmEvaluationWorkupthbulletrevisedfromLPtoruleoutCNSdisease;followwithITprophylaxisifclinicallyindicated"to"AllpatientsrequireadiagnosticLPatthetimeofinitialdiagnosisatdiseaserelapseoranyothertimewhenthereisaclinicalsuspicionforCNSinvolvementFollowwithITtreatmentprophylaxisasclinicallyindicated(seeBPDCN-B)."BPDCN-B-EVALUATIONANDTREATMENTOFCNSDISEASE•RecommendationsaddedfortreatmentwithandwithoutCNSdisease•Comprehensivepathologyreport,includingdiagnosisofAMLwithrecurrentcytogeneticsvs.AMLNOS,blastcount,cellularity,morphologicdysplasia,andmutationstatusifavailable•Humanleukocyteantigen(HLA)typingforpatientwithpotentialhematopoieticcelltransplantation(HCT)•Comprehensivepathologyreport,includingdiagnosisofAMLwithrecurrentcytogeneticsvs.AMLNOS,blastcount,cellularity,morphologicdysplasia,andmutationstatusifavailable•Humanleukocyteantigen(HLA)typingforpatientwithpotentialhematopoieticcelltransplantation(HCT)inthefuture(exceptforpatientswithamajorcontraindicationtoHCT)and/orearlyreferraltotransplantcenterBrainCTwithoutcontrastifcentralnervoussystemhagesuspectedbSeeAMLBBrainMRIwithcontrast,ifleukemicmeningitisedbSeeAMLBcionforextramedullaryMLB•Lumbarpuncture(LP),ifsymptomaticb(category2Bforasymptomatic)•Evaluatemyocardialfunction(echocardiogramorMUGAscan)inpatientswithahistoryorsymptomsofcardiacdiseaseorprior/plannedexposuretocardiotoxicdrugsorradiationtothorax•Considerearlyintegrationofpalliativecarec(SeeinesforPalliativeCarecationdationstificationandmendationsMLelinesforplasticsAcuteaNCCNGuidelinesVersion1.2022AcuteMyeloidLeukemia(Age≥18years)dexEVALUATIONFORAML•Historyandphysical(H&P)•Completebloodcount(CBC),platelets,differential,comprehensivemetabolicpanel,uricacid,lactatedehydrogenase(LDH)•B12andfolicacidevaluation•Prothrombintime(PT),partialthromboplastintime(PTT),fibrinogen•Bonemarrow(BM)corebiopsyandaspirateanalyses,includingimmunophenotypingbyimmunohistochemistry(IHC)stains+flowcytometryandcytogeneticanalyses(karyotype+FISH)(SeeAML-A)•Molecularanalyses(ASXL1,c-KIT,FLT3[ITDandrmutationsaSeeAMLATKD],NPM1rmutationsaSeeAMLADIAGNOSISd,eDIAGNOSISd,e,f,gAcutepromyelocyticleukemia(APL):ntswithclinicalorpathologicfeaturesSTUDIES(WHO2016)etinoicacidATRAetinoicacidATRAuponfirstsuspicionofAPL.hEarlyinitiationofventthelethalcomplicationofbleedinghIfcytogeneticandmoleculartestingtmentasforAMLToappropriatelystratifyavailableintensivetherapyoptions,expeditetestresultsofmolecularandcytogeneticanalysesforimmediatelyactionablemutationsorchromosomalabnormalities(eg,CBF,FLT3ary[ITDandTKD],NPM1,IDH1,IDH2aryic•Forpatientswithiciesdeuncontrolledwithiesdeleukapheresis,onedoseofintermediate-dosecytarabine(1–2g)maybeconsideredpriortoreceivingdiagnosticresults•Forpatientswhoprefernottoreceivebloodtransfusion(s),seeAML-DforgeneralconsiderationsandsupportivecareForsuspicionofblasticplasmacytoiddendriticcellneoplasm(BPDCN),seeBPDCN-INTROMyelodysplasticsyndromes(MDS)maeasticmaeAevaluationofBPDCNseeBPDCNNote:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.EVAL-1Version1.2022,12/02/21©2021NationalComprehensiveCancerNetwork(NCCN),Allrightsre