CMML诊治进展江苏省人民医院血液科洪鸣...Contents.Definition.WHOClassificationofMDS/MPN..Diagnosis.ClinicalmanifestationMDS-typeFatigueanddyspneaduetoanemiasusceptibilitytoinfectionsrarelybleedingMPN-typesignificantweightlossdrenchingnighsweatsleftupperquadrantpainfromsignificantsplenomegaly...Monocytosiswithmorphologicallynormalmonocytes(PB)MonocyteswithnuclearandCytoplasmicabnormalities(PB)CMML-1(BM)CMML-2(BM)RepresentativeperipheralbloodandBMsmearsdistinctionbetweenpromonocytesandabnormalmonocytesmaybeproblematicPromonocytestypicallyhavealight-graycytoplasmwithafewlilac-coloredgranulesandastipplednuclearchromatin.Abnormalmonocyteshavedenserchromatin,nuclearconvolutionsandfoldsandamoregreyishcytoplasm...Histopathology..ChromosomalabnormalitiesNospecificcytogeneticalterationshavebeenidentifiedinpatientswithCMML.Someofthemorefrequentlyreportedrecurringabnormalitiesinclude:.Chromosomalabnormalities.Chromosomalabnormalities110/414(27%)patientshadcytogeneticabnormalitiesMultivariableanalysisSurvivalandProgressiontoAMLLow-risk:normalor-YasasingleanomalyOSat5years:35%Intermediate-risk:allotherabnormalitiesOSat5years:26%high-risk:trisomy8orabnormalitiesofchromosome7orcomplexkaryotypeOSat5years:4%SuchE,CerveraJ,CostaD,etal.Cytogeneticriskstratificationinchronicmyelomonocyticleukemia.Haematologica.2011;96(3):375-383..MyelomonocyticClonalproliferationDiseaseprogressionSomaticmutations.SpliceosomalmutationsYoshida,etal.Frequentpathwaymutationsofsplicingmachineryinmyelodysplasia.Nature2011;478(7367):64-9.Lessconspicuouslybutsignificantly,SRSF2mutationsweremorefrequentinCMMLcases.MeggendorferM,etal.SRSF2mutationsin275caseswithchronicmyelomonocyticleukemia(CMML).Blood.2012Oct11;120(15):3080-8..＞3months＞1×109/L.CMML-1:blast(includingpromonocytes)<5%inPBand<10%inBMCMML-2:blastsfrom5%~19%inPBand10%~19%inBMorAuerrodsarepresentirrespectiveofblastcount...Diagnosticwork-up..Riskstratification..RiskstratificationMDAPS(M.D.AndersonPrognosticScore).OnepointforeachofthefollowingvariablesALC:absolutelympcytecountIMC:immaturemyeloidcells.RiskmodelsubgroupsscoreMediansurvival(months)low0-124Intermediate-1215Intermediate-238high45.NewMDSmodelappliedinCMMLwithleukocytosis(WBC＞12×109/L).Score.LevelsofrisklowInt-1Int-2high.Therapeuticoptions..Cytotoxicchemotherapy.Hypomethylatingagents.Allogeneicstemcelltransplantation(retrospectiveregistryfromlargetransplantcenters).cytotoxicchemotherapyHydroxyurearemainsthecornerstoneoftherapyPatientswithelevatedWBCcount(≥13×109/L)Hypomethylat-ingagentsORRsvaryfrom40to70%inselectedgroupsofpatientsApprovedbyFDAClinicaltrials.thebestoptionforpatientswhoarewillingtoparticipate.Allo-SCTRRshaverangedfrom17to50%andtreatment-relatedmortalityfrom12to52%.Patientsuptotheageof65-70withacompatibledonorRIChasbeenshowntoimproveNRM,OS,andRFSNovelagentsaloneorincombinationwithhypomethylatingagentsnucleosideanalog,immunomodul-atoryagent,andhistonedeacetylaseinhibitorsBeingactivelyinvestigated.ThanksforyourAttention！.
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