骨髓增生性腫瘤2022.v3-NCCN（英文版）

NCCNClinicalPracticeGuidelinesinOncologyNCCNGuidelines?)MyeloproliferativeNeoplasmsNCCNGuidelinesforPatients?availableat/patientsVersion3.2022,08/11/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon8/15/20229:20:22AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.lasmsdex*AaronT.Gerds,MD,MS/Chair??TCaseComprehensiveCancerCenter/UniversityHospitalsSeidmanCancerCenterandClevelandClinicTaussigCancerInstitute*JasonGotlib,MD,MS/Vice-Chair?StanfordCancerInstituteHarisAli,MD?ξCityofHopeNationalMedicalCenter*PrithvirajBose,MD?TheUniversityofTexasAndrewDunbar,MD?MemorialSloanKetteringCancerCenterAmroElshoury,MD?RoswellParkComprehensiveCancerCenterTracyI.George,MD≠HuntsmanCancerInstituteattheUniversityofUtahKrishnaGundabolu,MBBS?Fred&PamelaBuffettCancerCenter*ElizabethHexner,MD?ξAbramsonCancerCenterheUniversityofPennsylvaniaGabrielaS.Hobbs,MD?MassachusettsGeneralHospitalCancerCenteresPanelDisclosures*TaniaJain,MBBS?TheSidneyKimmelComprehensiveCancerCenteratJohnsHopkins*CatrionaJamieson,MD,PhD?UCSanDiegoMooresCancerCenterPaulR.Kaesberg,MD?UCDavisComprehensiveCancerCenterAndrewT.Kuykendall,MD?TMoffittCancerCenterYazanMadanat,MD?UTSouthwesternSimmonsComprehensiveCancerCenterBrandonMcMahon,MD?UniversityofColoradoCancerCenterSanjayR.Mohan,MD?Vanderbilt-IngramCancerCenterKalyanV.Nadiminti,MD?UniversityofWisconsinCarboneCancerCenterStephenOh,MD,PhD?SitemanCancerCenteratBarnes-JewishHospitalandWashingtonUniversitySchoolofMedicineAnimeshPardanani,MBBS,PhD?MayoClinicCancerCenterNikolaiPodoltsev,MD,PhD?YaleCancerCenter/SmilowCancerHospitalLindsayRein,MD?DukeCancerInstituteRachelSalit,MD?FredHutchinsonCancerResearchCenter/SeattleCancerCareAllianceBradyL.Stein,MD,MHS?TRobertH.LurieComprehensiveCancerCenterofNorthwesternUniversityMosheTalpaz,MD?UniversityofMichiganRogelCancerCenterPankitVachhani,MD?O'NealComprehensiveCancerCenteratUABMarthaWadleigh,MD??Dana-Farber/BrighamandWomen’senterSarahWall,MD,MPH?TheOhioStateUniversityComprehensiveCancerCenter-JamesCancerHospitalandSoloveResearchInstituteDawnC.Ward,MD≠UCLAJonssonComprehensiveCancerCentererPhD?Hematology/HematologiconcologyTInternalmedicine?Medicaloncology≠PathologyξTransplantation*DiscussionWritingCommitteeMemberVersion3.2022,08/11/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.?2017WHODiagnosticCriteriaforPrimaryMyelofibrosis(MPN-A)?IWG-MRTDiagnosticCriteriaforPostPV/PostETMF(MPN-B)?2017WHODiagnosticCriteriafor?2017WHODiagnosticCriteriaforPrimaryMyelofibrosis(MPN-A)?IWG-MRTDiagnosticCriteriaforPostPV/PostETMF(MPN-B)?2017WHODiagnosticCriteriaforPVandET(MPN-C)?PrognosticSignificanceofMutationsinMPN(MPN-D)?AssessmentofSymptomBurden(MPN-E1of2)?MyeloproliferativeNeoplasmsSymptomAssessmentForm:TotalSymptomScore(MPN-SAFTSS;MPN-10)(MPN-E2of2)?SupportiveCareforPatientswithMPN(MPN-F)?SpecialConsiderationsfortheUseofJAKInhibitors(MPN-G)?SpecialConsiderationsintheTreatmentofPVandET(MPN-H)?DefinitionofResistance/IntolerancetoHydroxyurea(MPN-I)lasmsdexiferativeNeoplasmsPanelMembersryoftheGuidelinesUpdatesMyeloproliferativeNeoplasms:?Workup(MPN-1)?DiagnosisandRiskStratification(MPN-2)Myelofibrosis:?TreatmentforLower-RiskMyelofibrosis(MF-1)?TreatmentforHigher-RiskMyelofibrosis(MF-2)?ManagementofMF-AssociatedAnemia(MF-3)?DiseaseProgressiontoAdvanced-Phase/AML(MF-4)?RiskStratificationforPatientswithMyelofibrosis(MF-A)?2013IWG-MRTANDELNResponseCriteriaforMF(MF-B)PolycythemiaVera:?TreatmentforLow-RiskPolycythemiaVera(PV-1)?TreatmentforHigh-RiskPolycythemiaVera(PV-2)?2013IWG-MRTandELNResponseCriteriaforPV(PV-A)?RiskStratificationforPatientswithPolycythemiaVera(PV-B)EssentialThrombocythemia:?TreatmentforVery-Low-RiskorLow-RiskET(ET-1)?TreatmentforIntermediate-RiskEssentialThrombocythemia(ET-2)?TreatmentforHigh-RiskEssentialThrombocythemia(ET-3)?2013IWG-MRTANDELNResponseCriteriaforET(ET-A)?RiskStratificationforPatientswithEssentialThrombocythemia(ET-B)Myelodysplastic/MyeloproliferativeNeoplasms(MDS/MPN):SeetheNCCNGuidelinesforMyelodysplasticSyndromesbelievesthatthebestnagementforanypatientwithcancerisinatrialoninclinicaltrialsisespeciallyencouragednsNCCNCategoriesofEvidenceandConsensus:Allrecommendationsarecategory2Aunlessotherwiseindicated.SeeNCCNCategoriesofEvidenceandConsensus.NCCNCategoriesofPreference:Allrecommendationsareconsideredappropriate.SeeNCCNCategoriesofPreference.TheNCCNGuidelinesareastatementofevidenceandconsensusoftheauthorsregardingtheirviewsofcurrentlyacceptedapproachestotreatmentAnyclinicianseekingtoapplyorconsulttheNCCNGuidelinesisexpectedtouseindependentmedicaljudgmentinthecontextofindividualstancestodetermineanypatientscareortreatmentTheNationalComprehensiveCancerNetworkNCCNmakesnorepresentationsorwarrantiesofanykindregardingtheircontentuseorapplicationanddisclaimsanyresponsibilityfortheirapplicationoruseinanywayTheNCCNationalComprehensiveCancerNetworkAllrightsreservedTheNCCNGuidelinesandtheillustrationshereinmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.?2022.Version3.2022,08/11/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.andmonitorfordiseaseprogressionforpatientswithsymptomaticlower-continuedneartothestartofconditioningtherapyfortheimprovementofriskMFwithnoresponseorlossofresponse.splenomegalyandotherdisease-relatedsymptomsandmonitorfordiseaseprogressionforpatientswithsymptomaticlower-continuedneartothestartofconditioningtherapyfortheimprovementofriskMFwithnoresponseorlossofresponse.splenomegalyandotherdisease-relatedsymptoms...(AlsoforMF-4).?Footnote"f"modified:ClinicalbenefitmaynotreachthethresholdoftheMF-3IWGResponseCriteriaandcontinuationofruxolitinibJAKinhibitorsis?Footnote"n"modified:RuxolitiniborfedratinibJAKinhibitorsmaybepriorJAKinhibitorforpatientswithhigher-riskMFwhoarenottransplantlimiteddataregardingtheuseoffedratiniborpacritinibwithHMAs.correspondingtoAlternateoptionnotusedbeforeandmonitorfordisease?Pacritinibhasbeenaddedto"SpecialConsiderationsfortheuseofJAKInhibitors".Footnotefmodified:Clinicalbenefitmaynotreachthethresholdoftherecommendedbasedonthediscretionofthecliniciancontinuedfortheimprovementofsplenomegalyandotherdisease-relatedfootnotecorrespondingtoAlternateoptionnotusedforinitialtreatment?Modifiedfootnote"k":RuxolitiniborfedratinibJAKinhibitorsmaybe?Considerpacritinibforpatientswithplateletcounts≥50x109/Lwithoneofsplenomegalyorotherdisease-relatedsymptoms.ThereareverycandidatesandwithnoresponseorlossofresponseisanewfootnoteMPN-G(5of7)andMPN-G(6of7)progression.lasmsdexsionoftheNCCNGuidelinesforMyeloproliferativeNeoplasmsfromVersionincludeMPN-F1of3?Sub-bullet1underVaccinations:Considerrecombinant(killed)zostervaccineforpatientson,ruxolitinibandfedratiniborpriorto,treatmentwithaJAKinhibitor.MS-1?Thediscussionsectionhasbeenupdatedtoreflectthechangesinthealgorithm.UpdatesinVersion2.2022oftheNCCNGuidelinesforMyeloproliferativeNeoplasmsfromVersion1.2022include:?Considerpacritinibforpatientswithplateletcounts<50x109/L,isa?Considerpacritinibforpatientswithplateletcounts<50x109/L,isanewinhibitorsisrecommendedbasedonthediscretionoftheclinician...?Pacritinibaddedasa?PacritinibaddedasatreatmentoptionforpatientswhoarenotMF-4recommendationusedincombinationwithHMA(azacitidineordecitabine)forthepalliationtransplantcandidateswithplatelets<50x109/L.Thisisacategory2ArecommendationusedincombinationwithHMA(azacitidineordecitabine)forthepalliationsionoftheNCCNsionoftheNCCNGuidelinesforMyeloproliferativeNeoplasmsfromVersioninclude?Bullet8,modified:Moleculartesting(bloodorbonemarrow)?Columnmutationalprognostication,isanewsub-bullet.?"m"modified:Erythropoiesis-mutationalprognostication,isanewsub-bullet.odelsincorporatingothermutationshavebeen?"n"Prostatecancerscreeningformenproposedtoidentifypatientsproposedtoidentifypatientswithmyelofibrosis(MF)aswellasPVandETtobettertobetterestimateoverallsurvivalmyelofibrosisfreesurvivalPVandETwhomaybeatriskofandratesofleukemictransformation?"u",modifiedwhomaybeatriskofandratesofleukemictransformationPlatelets≥50XPlatelets≥50X109/L,NotatransplantcandidateMF-A1of5?PrimaryMFMyelofibrosis(PMF)ommendationfromacategoryBommendationfromacategoryBUPDATESVersion3.2022,08/11/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.UPDATESVersion3.2022,08/11/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon8/15/20229:20:22AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.lasmsdexionoftheNCCNGuidelinesforMyeloproliferative?Ropeginterferonalfa-2b-njft:pisacategory2Brecommendationforlow-riskpolycythemiaveraunderotherrecommendedregimen.?Column5,Bullet2,modified:Frequentphlebotomyand/orpersistentneedforphlebotomy,butwithpoortoleranceintolerantofphlebotomy(AlsoforPV-2).?Ropeginterferonalfa-2b-njft:pColumn2:otherrecommendedregimenforhigh-riskploycythemiaverapColumn6:ifnotpreviouslyused,underotherrecommendedregimen?Ruxolitinib(category1)recommendationforhigh-riskPVfroma(category2A)?Deleted:Busulfan(PO)(category2B)(especiallyforolderadults)Usefulincertaincircumstances(alsoforET-4).?"j":modifiedtoincludetherapythatdeferhydroxyureaorropeginterferonalfa-2b-njft.?Deleted:AlvarezLarranA,etal.AnnHematol2014;93:2037-2043.(AlsoforET-4)NewpageforMIPSS-PVriskstratificationwiththefollowingreference:TefferiA,GuglielmelliP,LashoTL,etal.Mutation-enhancedinternationalprognosticsystemsforessentialthrombocythaemiaandpolycythaemiavera.BrJHaematol.2020;189:291-302.ET-1?Addedthefollowingto"very-low-risk":(Age≤60years,noJAK2mutation,nopriorhistoryofthrombosis)p"and/orlow-riskJAK2-positivepatients"wasremoved.?Addedthefollowingto"low-risk":(Age≤60years,withJAK2mutation,nopriorhistoryofthrombosis)p"forpatientswithvasomotor/microvasculardisturbancesand/orlow-riskJAK2-positivepatients"wasremoved.ET-2?AddedthefollowingtoIntermediate-risk(Age>60years,noJAK2mutation,nopriorhistoryofthrombosis)p"forvasomotor/microvasculardisturbances"wasremoved.?AddedthefollowingtoHigh-risk(Historyofthrombosisatanyage;or,age>60yearswithJAK2mutation)?Column2,Bullet1,modifiedtoinclude"oranagrelide".ioninclude?NewpageforMIPSS-ETriskstratificationwiththefollowingreference:TefferiA,GuglielmelliP,LashoTL,etal.Mutation-enhancedinternationalprognosticsystemsforessentialthrombocythaemiaandpolycythaemiavera.BrJHaematol.2020;189:291-302.MPN-F(1of3)?Bullet8,sub-bulletsdeleted:pHydrationand/ordiuresispConsidermanagementofhyperuricemiawithallopurinolorrasburicase.pRasburicaseshouldbeconsideredasinitialtreatmentinpatientswithrapidlyincreasingblastcounts,highuricacid,andevidenceofimpairedrenalfunction.MPN-H(1of4)?Bullet1,sub-bullet2,modified:Consideraspirinforpatientswithothercardiovascularriskfactors.Therisksandbenefitsofaspirinplusanticoagulationneedtobeindividualizedonacase-by-casebasis.(referstoallsubtypes)(SeeET-1andET-2).pSub-bullet3,3rdsentence,modified:CautionisrequiredwhenusingantiplateletagentswithanticoagulantsforthetreatmentofthrombosisthromboticdisordersinpatientswithPV.pSub-bullet4,3rdsentence,modified:Thevalueofcytoreductioninreducingfuturevasculareventshasnotbeenstudiedinaprospective,randomizedcontrolledtrialsinPV.?Bullet2,sub-bullet2,2ndsentence,modified:Considertheuseofappropriatecytoreductivetherapytooptimizenormalizeplateletcountswhileminimizinghematologicandnon-hematologictoxicities,seediscussion.?ReferenceupdatedtoZwickerJI,ParanagamaD,LessenDS,etal.Hemorrhageinpatientswithpolycythemiaverareceivingaspirinwithananticoagulant:Aprospective,observationalstudy.Haematologica2021Jun24.doi:10.3324/haematol.2021.279032.Onlineaheadofprint.MPN-H(2of4)and(3of4)?Terminologiesmodifiedtobemoreinclusiveofallsexualandgenderidentities.?Thesectionon"Pregnancy"hasbeensignificantlyedited.ReferencesHematologicmalignanciesinpregnancy:Managementguidelinesfromaninternationalconsensusmeeting.JClinOncol2016;34:501-508,isnewreferringto"DefinitionofHigh-RiskPregnancyinMPN".nosticationeSuspicionof rativeasmsMPNaPrintedbyMinTangon8/15/20229:20:22AM.FornosticationeSuspicionof rativeasmsMPNalasmsdexWORKUP?H&P,includingspleensizebypalpation,evaluationofthrombotic/hemorrhagiceventsandcardiovascularriskfactors?CBCwithdifferential?Comprehensivemetabolicpanelwithuricacid,lactatedehydrogenase(LDH),andliverfunctiontests(LFTs)?FISHormultiplexRT-PCR(ifavailable)forBCR-ABL1toexcludethediagnosisofCML;ifBCR-ABL1-positive,SeeNCCNGuidelinesforChronicMyeloidLeukemia?Examinationofbloodsmearstainbcd?Bonemarrowaspiratewithironstain;bonemarrowbiopsywithstainbcdBonemarrowcytogeneticsblood,ifbonemarrowisinaspirable)(karyotypewithorwithout?Moleculartesting(bloodorBonemarrowcytogeneticsblood,ifbonemarrowisinaspirable)(karyotypewithorwithoutwithPV)ormoleculartestingusingmultigeneNGSpanelthatincludesJAK2,CALR,andMPLeandMPLmutationswithPV)ormoleculartestingusingmultigeneNGSpanelthatincludesJAK2,CALR,andMPLeIfthereisevidenceofmastIfthereisevidenceofmastcellaggregatesinthebonemarrow,SeeNCCNGuidelinesfor?AssessmentofsymptomburdenusingMPNSymptomAssessmentFormTotalSymptomScore(MPN-SAFTSS;MPN-10;MPN-E2of2)?Documentationoftransfusion/medicationhistory?Humanleukocyteantigen(HLA)testing,ifconsideringallogeneichematopoieticcell?Serumerythropoietin(EPO)level?Serumironstudies?uutisneuiredvonWillebranddisease(VWD)and/orotherpProthrombintime(PT),partialthromboplastintime(PTT),fibrinogenSeeWorkupintheNCCNGuidelinesforSystemicePrognosticmodelsincorporatingotherSeeWorkupintheNCCNGuidelinesforSystemic(MF)aswellasPVandETtobetterestimateoverallsurvival,myelofibrosis-freesurvival(PVandET),andloidLymphoidNeoplasmswithratesofleukemictransformation.Next-generationsequencing(NGS)loidLymphoidNeoplasmswithhiliaandTyrosineKinaseFusionGenesbSeeWHODiagnosticCriteriaforPrimaryselectedcircumstanceshiliaandTyrosineKinaseFusionGenesbSeeWHODiagnosticCriteriaforPrimarySeeMPN-cd)WHODiagnosticCriteriaforPVandET.Seemaybeusefulundercertaincircumstances.maybeusefulundercertaincircumstances.Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.MPN-1Version3.2022,08/11/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon8/15/20229:20:22AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.lasmsdexDIAGNOSISi,jMyelofibrosisPolycythemiavera(PV)dthrombocythemiaET)FootnotesonMPNAPROGNOSTICRISKMODELPrimarymyelofibrosis(PMF)b?MIPPS-70orMIPSS-70+Version2.0(preferred)?DIPSS-Plus(ifmoleculartestingisnotavailable)or?DIPSS(ifkaryotypingisnotavailable)Post-PVorPost-ETMFc?MYSEC-PMnalriskmodelksisrevisedlRISKSTRATIFICATIONLower-risk(MF-1)?MIPSS-70:≤3?MIPSS-70+Version2.0:≤3?DIPSS-Plus:≤1DIPSS≤2MYSECPM<14Higher-risk(MF-2)?MIPSS-70:≥4?MIPSS-70+Version2.0:≥4?DIPSS-Plus:>1DIPSS>2MYSECPM≥14Low-risk(PV-1)?Age<60yearsandnopriorhistoryofthrombosisHigh-risk(PV-2)?Age≥60yearsand/orpriorhistoryofthrombosisVery-low-risk(ET-1)?Age≤60years,noJAK2mutation,nopriorhistoryofthrombosisLow-risk(ET-1)?Age≤60years,withJAK2mutation,nopriorhistoryofthrombosisIntermediate-risk(ET-2)?Age?60years,noJAK2mutation,nopriorhistoryofthrombosisHigh-risk(ET-3)?Historyofthrombosisatanyageorage>60yearswithJAK2mutationNote:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.MPN-2Version3.2022,08/11/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.PrintedbyMinTangon8/15/20229:20:22AM.Forpersonaluseonly.Notapprovedfordistribution.Copyright?2022NationalComprehensiveCancerNetwork,Inc.,AllRightsReserved.lasmsdexbSee2017WHODiagnosticCriteriaforPrimaryMyelofibrosis.See(MPN-A).cDiagnosticcriteriaforpost-ETorpost-PVMF.See(MPN-B).dSee2017WHODiagnosticCriteriaforPVandET.See(MPN-C).iThediagnosisofMPNisbasedonthe2017WHOCriteriaandrequiresacombinationofclinical,laboratory,cytogenetic,andmoleculartests.jReferraltospecializedcenterswithexpertiseinthemanagementofMPNisstronglyrecommendedforallpatientsdiagnosedwithMF,PV,orET.kMarchioliR,etal.JClinOncol2005;23:2224-2232.lTherevisedInternationalPrognosticScoreofThrombosisforET(IPSET-thrombosis)ispreferredfortheriskstratificationofET(HaiderM,etal.AmJHematol2016;91:390-394.BarbuiT,etal.BloodCancerJ2015;5:e369).Note:Allrecommendationsarecategory2Aunlessotherwiseindicated.ClinicalTrials:NCCNbelievesthatthebestmanagementofanypatientwithcancerisinaclinicaltrial.Participationinclinicaltrialsisespeciallyencouraged.MPN-2AVersion3.2022,08/11/22?2022NationalComprehensiveCancerNetwork(NCCN),Allrightsreserved.NCCNGuidelinesandthisillustrationmaynotbereproducedinanyformwithouttheexpresswrittenpermissionofNCCN.Lower-riska(MPN-10;rialincircumstances:Ruxolitinibssymptomsse Peginterferonalfa-2aNEofevery3–6reaifLower-riska(MPN-10;rialincircumstances:Ruxolitinibssymptomsse Peginterferonalfa-2aNEofevery3–6reaifctionwouldaticallyldexTREATMENTFORLOWER-RISKMYELOFIBROSISContinueobservation(if?symptomaticContinueobservation(if?symptomaticandmonitorforandsymptomsondiseaseprogressionondiseaseprogressionc(MPN- Asymptomatic Asymptomaticrialomaticpatientsshould?(MPN-10;rialomaticpatientsshould?edasnotedbelowAssessedasnotedbelowAssesssymptomburdenusingMPN-SAFTSSResponseorLossDiseaseprogressiongContinuetreatmentiseaseprogressionciseaseprogressioncefMPNMPNEof)AlternateoptionnotusedforinitialtreatmentandefefhMPNMPNEof)Higher-riskseeMF-2;andAdvancedstageMF/AML,seeMF-4aEvaluationforallogeneicHCTisrecommendedforpatientswithlowplateletcountsorcomplexcytogenetics.Identificationof“higher-risk”mutationsmaybehelpfulinthedecision-makingregardingallogeneicHCTforpatientswithPMF.SeePrognosticSignificanceofMutationsinMPN(MPN-D).eSeeSpecialConsiderationsfortheUseofJAKInhibitors(MPN-G).beSeeSpecialConsiderationsfortheUseofJAKInhibitors(MPN-G).fClinicalbenefitmaynotreachthethresholdoftheIWGResponsefClinicalbenefitmaynotreachthethresholdoftheIWGResponseCriteriaandcontinuationofJAKinhibitorsisrecommendedbasedonthediscretionoftheclinician.See2013IWG-MRTandELNResponseCriteriaforMF(MF-B).gAdditionalmoleculartestingusingmulti-geneNGSpanelshouldbeconsideredtoevaluateforhigher-riskmutationsassociatedwithdiseaseprogressioninpatientswithprimaryPMF.SeePrognosticSignificanceofMutationsinMPN(MPN-D).asclinicallyindica