九章白血病专业知识讲座

九章白血病专业知识讲座九章白血病专业知识讲座第1页讲授目标和要求1.掌握急、慢性白血病临床表现，试验室检验及诊疗标准，治疗标准2.熟悉急性白血病FAB分型，联合化疗标准，完全缓解概念九章白血病专业知识讲座第2页讲授主要内容概述病因和发病机制临床表现试验室检验诊疗标准判别诊疗治疗九章白血病专业知识讲座第3页Erythrocytes:transportoxygenNeutrophilBasophilEosinophilMonocytes/MacrophageDefenseagainstinfectionPlatelets:MediatebloodclottingT-lymphocytes:antigenpresentingB-lymphocytesPlasmacell:Sourceofantibodies九章白血病专业知识讲座第4页PluripotentialstemcellsMyeloidstemcellsLymphoidstemcellsUnipotentialprogenitorcellsImmaturehematopoieticcellsmaturehematopoieticcells九章白血病专业知识讲座第5页Hematopoiesiscomposesoftheoptionsofcommitmenttodifferentlineagesandtheprogressivestagesofmaturationatwhichpartialorcompletearrestcanoccur,resultsinthewidearrayofmalignantdisease-LeukemiaStemcellProgenitorcellImmaturecellMaturecell九章白血病专业知识讲座第6页AccumulationofmutationsofDNAwithinapluripotentialstemcellorveryearlyprogenitorcellgivesrisetoleukemicstemcellsNormalstemcellLeukemicstemcell九章白血病专业知识讲座第7页九章白血病专业知识讲座第8页九章白血病专业知识讲座第9页Etiology&PathogenesisEnvironmentalfactors

AcquireddiseasesLesionstotheDNAClonalexpansion九章白血病专业知识讲座第10页Alotofenvironmentalfactorshasbeenreportedtocauseleukemia.However,onlyfourofthemarefirmlyestablishedcausalagents.Theyare:IrradiationexposureChronicbenzeneexposureChemotherapeuticagentsLeukemiavirusinfectionEnvironmentalfactorscauseleukemia九章白血病专业知识讲座第11页九章白血病专业知识讲座第12页Inheritedsyndromessuchasataxia-telangiectasia,downsyndromepredisposetosubsequentdevelopmentofleukemia.Usually,thesekindsofsyndromessharethecommonfeaturesthattheyallhavehereticdefectsintheirgenomegavebytheirparentsInheritedsyndromespredisposetoleukemia九章白血病专业知识讲座第13页AcquireddiseasepredisposetoleukemiaLeukemiamayalsodevelopfromtheprogressionofotherclonaldisordersofhematopoieticstemcells.Ploycythemiavera,idiopathicmyelofibrosis,etc九章白血病专业知识讲座第14页LeukemiaClassificationThereareatleastdozensofvarietiesofleukemia.Theyareclassifiedbyhowquicklyitprogresses.Acuteleukemiaisfast-growingandcanoverrunthebodywithinafewweeksormonths.ByContrast,chronicleukemiaisslow-growingandprogressivelyworsenoveryears九章白血病专业知识讲座第15页AcuteversuschronicleukemiaAcute:thebloodcellsofacuteleukemiaremaininanimmaturestate,sotheyreproduceandaccumulateveryrapidly.Therefore,theyneedtreatmentimmediately,otherwisethediseasemaybefatalwithinfewmonthsChronic:inChronicleukemia,thebloodcellseventuallymature,orpartiallymature.Buttheyarenot“normal”.Theyremaininthebloodmuchlongerthannormalbloodcellsandtheycannotactfunctionalcellswell九章白血病专业知识讲座第16页MyelogenousversuslymphocyticleukemiaIftheleukemiccellsarisefrommyeloidpluripotentialstemcells:myeloidleukemiaIftheleukemiccellsarisefromlymphocyticpluripotentialstemcells:lymphocyticleukemia九章白血病专业知识讲座第17页ClinicalmanifestationsLeukemichematopoiesisNormalhematopoiesismarrowfailureInfiltration九章白血病专业知识讲座第18页MarrowfailureAnemia(lossoferythocytes):fatigues,pallorweakness,reducedexercisetoleranceFeverandinfection(Poorinfectionfighters)Abnormalbleeding(lossofplatelets)九章白血病专业知识讲座第19页InfiltrationsOraltissue:swollenpainful,andbleedinggumsSplenomegalyandhepatomegalyLymphnodeenlargementBoneorjointpainCNS-headaches,seizures,weakness,blurredvisionandvomiting九章白血病专业知识讲座第20页九章白血病专业知识讲座第21页

BloodtestfindingsAnemiaisaconstantfeature.Nucleatedredcellsorimmatureredbloodcellmaybepresent.Thrombocytopeniaisnearlyalwayspresentatthetimeofdiagnosis.Thetotalleukocytecountscanbehigh,normalorlow.

Immaturehematopoieticcellsarealmostpresentintheblood九章白血病专业知识讲座第22页MarrowfindingsNormalbonemarrowAMLmarrow九章白血病专业知识讲座第23页Cytogeneticfindings九章白血病专业知识讲座第24页Diagnosis&ClassificationOthernewlydevelopedmethods九章白血病专业知识讲座第25页Morphology:thebonemarrowcellsareevaluatedaccordingtotheirsize,shape,andcontentofgranulesandthentheyareclassifiedwithrespectedtomaturityCytochemistrystaining:identificationofthechemicalcomponentsofcellsisconductedtodistinguishdifferenttypesofleukemia.Cytochemistryoftenusespecialcoloreddyes九章白血病专业知识讲座第26页AcuteleukemiaAMLALLM0:undifferentiatedAMLM1:Myeloblasticleukemia(withoutmaturation)M2:Myeloblasticleukemia(withmaturation)M3:promyelocyticleukemiaM4:MyelomonocyticleukemiaM5:MonocyticleukemiaM6:ErythroleukemiaM7:MegkaryoblasticleukemiaL1:MatureappearinglymphoblastsL2:ImmatureandvariouslyshapedlymphoblastsL3:Lymphoblastsarelargeanduniform九章白血病专业知识讲座第27页九章白血病专业知识讲座第28页P142(CDtables)AlotofCDprovidescluesforthediagnosis九章白血病专业知识讲座第29页FlowCytometryImmunohistochemistry九章白血病专业知识讲座第30页

ImmnuophenotypingpanelusedinSt.JudeChildren’sresearchhospitalU.S.A.CD13CD33CD19CytoCD79aCD7CytoCD3AML----B-ALL----T-ALL----Byusingthismethodofanalysis,onecanmakeafirmdiagnosisin99%ofcases九章白血病专业知识讲座第31页免疫表型分型方案T细胞B细胞（4%）B细胞前体

CD7（敏感），cCD3（特异）CD19(敏感),cCD79a(特异)成熟T细胞（18%）前T细胞（6%）前B-细胞（9%）早期前-B细胞（52%）前-前-B细胞（11%）sIg,sIg九章白血病专业知识讲座第32页九章白血病专业知识讲座第33页Inserttable90%ofthecaseswithleukemiahavenon-randomizedtranslocation九章白血病专业知识讲座第34页P118typesoftranslocationsCMLAML-M2AML-M3AML-M4AMLAML-M4E0九章白血病专业知识讲座第35页九章白血病专业知识讲座第36页九章白血病专业知识讲座第37页九章白血病专业知识讲座第38页Othernewdevelopedmethods九章白血病专业知识讲座第39页DifferentialDiagnosisPseudoleukemiaMyelodysplasticsyndromeNonleukemicpancytopenia九章白血病专业知识讲座第40页TreatmentSupportivecaresandpreparationofthepatientsAntileukemictherapyTherapyofthecentralnervoussystemStemcelltransplantation九章白血病专业知识讲座第41页AnemiaHemorrhageInfection九章白血病专业知识讲座第42页

HematologicalsupportTransfusionofPlateletsTransfusionofpackedredcellsTransfusionsofgranulocytesTransfusionsofIgG九章白血病专业知识讲座第43页

InfectioncontrolSpecialprecautions:protectiveisolationEliminationofcontaminatedfoodsOralanddigestivesystemcareBroadspectrumantibioticsGM-CSFadministration九章白血病专业知识讲座第44页AntileukemictherapyChemotherapytokillleukemiacellsusingstronganti-cancerdrugs九章白血病专业知识讲座第45页TreatmentphasesInductiontherapy:theaimistobringaboutremission,thatisleukemiccellsarenolongerfoundinthebonemarrowandtherecoveryofnormalhematopoiesisPost-remissiontherapy:toeliminateanyleukemiacellspotentialhidinginthebody九章白血病专业知识讲座第46页SpecialsubtypesAcutepromyelocyticLeukemia(APL):becauseofthesmallparticles(procoagulants)insidetheAPLcell,DICarecommonlyseeninthistypeofAML九章白血病专业知识讲座第47页NormalAPLGenesessentialfordifferentiationareshutdownbythefusionproteinsPML-RARalpha九章白血病专业知识讲座第48页Differentiationagents(all-transretinoicacid,arsenictrioxide)九章白血病专业知识讲座第49页Hyperleukecytosis:causeabatchofcomplicationsandshouldbetreatedusingleukapheresis(exchangingtransfusion)orpreinductiontreatmentpriortointensivechemotherapy九章白血病专业知识讲座第50页

Acuteleukemiaover60yearsofageislessresponsivetochemotherapyStandardtwo-drugstherapycanbeused.CR30%Intoleranttostandardtherapy.Low-doseofAra-Ccanbeusedfor14-28days九章白血病专业知识讲座第51页TherapyoftheCNSleukemiaTheCNSisafrequentsanctuarysiteforleukemiccellsbecausemostofthechemotherapydrugsarenotabletoreachtheCNS.SometypesofacuteleukemiahavetendencytobewithCNSleukemiasuchasAML(M4,M5),ALL九章白血病专业知识讲座第52页IntrathecalchemotherapyCranialirradiation九章白血病专业知识讲座第53页StemcelltransplantationsThemainpurposeofBMTandPBSCTincancertreatmentistomakeitpossibleforpatientstoreceiveveryhighdosesofchemoth