骨髓增生异常综合征诊治-英文2011PPT课件

Myelodysplasticsyndromes:ApracticalapproachtodiagnosisandtreatmentHong-lingHao,M.D.DepartmentofHematology,HebeiGeneralHospitalIntroductionMDSareaheterogeneousgroupofdisordersofbloodcellproductioninthebonemarrowthatcantransformintoacutemyeloidleukemia(AML).Theyarediagnosedmostoftenintheelderly.Theconsequentcytopeniasresultininfectionsandbleedingcomplications.Therangeofsymptomsdependsonthebonemarrowcelltypeaffected.Theyhavesymptomsstemmingfromanemiaorthrombocytopenia,orhaverecurrentinfections.SubtypesofMDShavedifferentpathologicandclinicalpresentationsanddifferentprognoses.Theyareoftencategorizedaslower-riskorhigher-risk,dependingonthelikelihoodoftransformingtoAML.Lower-riskMDSsurviveamedianof3to7years.Higher-risktypesarepathobiologicallysimilartoAMLinolderadults,andpatientseitherdevelopAMLordieofcomplicationsofMDS,onaveragewithin1.5years.EtiologyGeneticandenvironmentalfactorsprobablybothplayarole.Environmentalfactors:smoking;ionizingradiation;exposuretobenzene,hairdyes,pesticides.Geneticfactors:Downsyndrome,Fanconianemia,andBloomsyndromeareassociatedwithMDS.MDSrarelyruninfamilies.Diagnosis——LaboratorytestsCompletebloodcellcount(CBC)EvaluationofanemiaTestsfornutrientdeficienciesFecaloccultbloodtestingLiverfunctiontestsRenalfunctiontestsThyroidfunctiontestsReticulocytecountEPOlevelScreeningforrelevantinfections:HIV,Hepatitis,ParvovirusB19……Reviewofdrugs:MTX,Depakote,Luminal,Retrovir….EvaluationofothercytopeniasAbdominalultrasonography----forsplenomegalyCoombsantiglobulintestsLDHlevelAntinuclearantibodytiterRheumatoidfactorlevelIftestsarenegativePeripheralbloodsmearBonemarrowaspirationandbiopsySpecializedtests:1.Antiplateletantibodies2.Proteinelectrophoresis3.Fluorescenceinsituhybridization——FISHEachhasitsprognosticandtherapeuticimplications!ClassificationSystemsforMDSMDShavetwomainclassificationsystems,theFABandtheWHO.TheWHOclassificationiswidelyacceptedbecauseitincorporatesmorphologicandcytogeneticfactorsandcorrelateswithprognosis.FABclassificationforMDSWHOclassificationforMDS接上一页ScoringsystemsforMDSThesesystemsarebasedon:Thenumberofmyeloblastsinthebonemarrow.(Thehigherthecount,theworsetheprognosis.)Thenumberordegreeofcytopenias.Cytogeneticabnormalities.Themostwidelyusedprognosticsystems:IPSS:InternationalPrognosticScoringSystemWPSS:WHOClassification-basedPrognosticScoringSystemBlood,Vol89,No6(March15),1997:pp2079-2088CancerControl2008;15(suppl4):4–13.TreatmentofMDS1.SupportivecareIncludestransfusionofbloodproductstominimizecomplicationsofcytopeniasandtoimprovequalityoflife,aswellasantibioticstotreatactiveinfections.Redbloodcellstypicallyaregivenwhenthehemoglobinlevelfallsbelow8.5g/dL.Plateletsaregivenwhentheplateletcountisbelow10×109/L,intheabsenceofsymptoms.Ironchelation:Itisdebatable?Bloodproducttransfusionscanleadtoironoverload,particularlywithalifetimeadministrationofmorethan20units.Thisinterventionshouldbereservedforpatientswithlower-riskdiseasewhoareexpectedtosurvivemorethan1year.Antibiotics:Theriskofinfectionissignificantlyincreasedwhentheneutrophilcountisbelow0.5×109/L.Fever(temperature>38°C)inneutropenicpatientsisanemergency,requiringimmediateinitiationofbroad-spectrumantibiotics.ProphylacticantibioticshavenoprovenroleinMDSpatientswithneutropenia.2.Treatmentoflower-riskdisease(1)Erythropoiesis-stimulatingagents:Epo<100IU/Landhaveminimaltransfusionneeds.Additionofacolony-stimulatingfactorcanbeconsideredforpatientswithneutropenia.Responseismeasuredasanimprovementinhemoglobinorasindependencefromtransfusions.(2)Othertreatments:(IfESAtreatmentisineffective)Immunosuppressivetherapy:ATGLenalidomideforMDSwith5q-candecreasetheneedforbloodtransfusioninapproximately2/3ofthesepatients.AzacitidineordecitabineStemcelltransplantationExperimentaltreatments:clinicaltrials3.Treatmentofhigher-riskdiseaseAbout25%ofpatientswithnewlydiagnosedMDSand15%to20%ofpatientswithestablishedMDShavehigher-riskdisease.Regardlessofbloodcounts,giventhehighlikelihoodoftransformationtoAMLordeathwithin1.5years.Thetreatmentoptionsforhigher-riskdiseaseinclude:Methyltransferaseinhibitorssuchasazacitidineanddecitabine.Cytotoxicchemotherapy(similartotreatmentofacutemyeloidleukemia).Bonemarrow-hematopoieticstemcelltransplantation(HSCT).Experimentaltreatmentsinclinicaltrials.StemcelltransplantationcuresMDSHematopoieticstemcelltransplantationistheonlycurativetreatmentforMDS.However,itisperformedinfewerthan5%ofpatients,becausetherateoftransplant-relateddeathishigh.Thebalancebetweenrisksandbenefitsofthistreatmentsmustbejustifiable.Take-homePointsMDSarecomplexandheterogeneous,sotreatmentoptionsrangefromsupportivecaretochemotherapyandallogeneicstemcelltransplantation.Thankyouforyourattention!E-mail:h0707@163.comMP:18931891239OntheStorkTowerWangZhi-huanThesunbeyondthemountainsglows,TheYellowRiverseawardsflows.Youcanenjoyagrandersight,Byclimbingtoagreaterheight.