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1、HongzhiXuShandongProvincialHospitalMyeloproliferativeDisordersCONTENTSPathogenesisandmanagementofessentialthrombocythemiaIdiopathicerythrocytosis:adisappearingentityTherapeuticpotentialofJAK2inhibitorsPathogenesisandmanagementofessentialthrombocythemiaPathogenesisRelationshi
2、pofETtoPVandPMFThelevelofJAK2-STAT5signalingprovidesarheostatthatdetermineswhetherthediseasephenotypeispredominantlyerythroidormegakaryocytic.Severallinesofevidencesuggestablurringofthedistinctionbetweenthesedisorders.AproporationofpatientsdiagnosedwithET(seeTable1forcriteri
3、a)harborincreasedlevelsofbonemarrowreticulinintheabsenceofotherfeaturessuggestingadiagnosisofPMFThevariabledegreeofreticulinaccumulationreflectsthecombinedeffectsofgeneticbackground,diseaseduration,therapy,clonalburdenandtheacquisitionofadditionalgeneticlesions.Table1.Sugges
4、teddiagnosticcriteriaforessentialthrombocythemia(ET)DiagnosisrequiresA1-A3ORA1+A3-A5A1Sustainedplateletcount>450X109/L.A2Presenceofanacquiredpathogeneticmutation(eg,inJAK2orMPL).A3Noothermyeloidmalignancy,especiallypolycythemiavera(PV),primarymyelofibrosis(PMF),chronicmyeloi
5、dleukemia(CML)ormyelodysplasticsyndrome(MDS).A4Noreactivecauseforthrombocytosisandnormalironstores.A5Bonemarrowtrephinehistologyshowingincreasedmegakaryocyteswithprominentlargehyperlobatedforms;reticulinisgenerallynotincreased(≤2ona0-4scale).FamilialPredispositiontoETandOthe
6、rMyeloproliferativeNeoplasmsArelativeriskof7.4fordevelopingETinthosewithanaffectedfirst-degreerelative.AreMutationsinJAK2Disease-initiatingEvents?TheacquisitionofaJAK2mutationwasprecededbyeitheradeletionofchromosome20q24oramutationinTET2.Directevidencenowexistsdemonstratingt
7、hatJAK2mutationsarenotthedisease-initiatingeventinsomepatients,althoughthefrequencyofthisscenarioremainsunclear.ProgressiontoAcuteMyeloidLeukemiaProgressiontoacutemyeloidleukemia(AML)occursinasmallminorityofETpatientsandinvolvestheaccrualoffurthergeneticevents.DiagnosisandMa
8、nagementDiagnosticCriteriaMutationsinJAK2exon12arenotthoughttooccurinpatien
