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1、HongzhiXuShandongProvincialHospitalMyeloproliferativeDisordersCONTENTSPathogenesisandmanagementofessentialthrombocythemiaIdiopathicerythrocytosis:adisappearingentityTherapeuticpotentialofJAK2inhibitorsPathogenesisandmanagementofessentialthrombocythemiaPathogenesisRelations
2、hipofETtoPVandPMFThelevelofJAK2-STAT5signalingprovidesarheostatthatdetermineswhetherthediseasephenotypeispredominantlyerythroidormegakaryocytic.Severallinesofevidencesuggestablurringofthedistinctionbetweenthesedisorders.AproporationofpatientsdiagnosedwithET(seeTable1forcri
3、teria)harborincreasedlevelsofbonemarrowreticulinintheabsenceofotherfeaturessuggestingadiagnosisofPMFThevariabledegreeofreticulinaccumulationreflectsthecombinedeffectsofgeneticbackground,diseaseduration,therapy,clonalburdenandtheacquisitionofadditionalgeneticlesions.Table1.
4、Suggesteddiagnosticcriteriaforessentialthrombocythemia(ET)DiagnosisrequiresA1-A3ORA1+A3-A5A1Sustainedplateletcount>450X109/L.A2Presenceofanacquiredpathogeneticmutation(eg,inJAK2orMPL).A3Noothermyeloidmalignancy,especiallypolycythemiavera(PV),primarymyelofibrosis(PMF),chron
5、icmyeloidleukemia(CML)ormyelodysplasticsyndrome(MDS).A4Noreactivecauseforthrombocytosisandnormalironstores.A5Bonemarrowtrephinehistologyshowingincreasedmegakaryocyteswithprominentlargehyperlobatedforms;reticulinisgenerallynotincreased(≤2ona0-4scale).FamilialPredispositiont
6、oETandOtherMyeloproliferativeNeoplasmsArelativeriskof7.4fordevelopingETinthosewithanaffectedfirst-degreerelative.AreMutationsinJAK2Disease-initiatingEvents?TheacquisitionofaJAK2mutationwasprecededbyeitheradeletionofchromosome20q24oramutationinTET2.Directevidencenowexistsde
7、monstratingthatJAK2mutationsarenotthedisease-initiatingeventinsomepatients,althoughthefrequencyofthisscenarioremainsunclear.ProgressiontoAcuteMyeloidLeukemiaProgressiontoacutemyeloidleukemia(AML)occursinasmallminorityofETpatientsandinvolvestheaccrualoffurthergeneticevents.
8、DiagnosisandManagementDiagnosticCriteriaMutationsinJAK2exon12arenotthoughttooccurinpatien
