九章白血病專業(yè)知識(shí)講座

九章白血病專業(yè)知識(shí)講座九章白血病專業(yè)知識(shí)講座第1頁講授目標(biāo)和要求1.掌握急、慢性白血病臨床表現(xiàn)，試驗(yàn)室檢驗(yàn)及診療標(biāo)準(zhǔn)，治療標(biāo)準(zhǔn)2.熟悉急性白血病FAB分型，聯(lián)合化療標(biāo)準(zhǔn)，完全緩解概念九章白血病專業(yè)知識(shí)講座第2頁講授主要內(nèi)容概述病因和發(fā)病機(jī)制臨床表現(xiàn)試驗(yàn)室檢驗(yàn)診療標(biāo)準(zhǔn)判別診療治療九章白血病專業(yè)知識(shí)講座第3頁Erythrocytes:transportoxygenNeutrophilBasophilEosinophilMonocytes/MacrophageDefenseagainstinfectionPlatelets:MediatebloodclottingT-lymphocytes:antigenpresentingB-lymphocytesPlasmacell:Sourceofantibodies九章白血病專業(yè)知識(shí)講座第4頁P(yáng)luripotentialstemcellsMyeloidstemcellsLymphoidstemcellsUnipotentialprogenitorcellsImmaturehematopoieticcellsmaturehematopoieticcells九章白血病專業(yè)知識(shí)講座第5頁Hematopoiesiscomposesoftheoptionsofcommitmenttodifferentlineagesandtheprogressivestagesofmaturationatwhichpartialorcompletearrestcanoccur,resultsinthewidearrayofmalignantdisease-LeukemiaStemcellProgenitorcellImmaturecellMaturecell九章白血病專業(yè)知識(shí)講座第6頁AccumulationofmutationsofDNAwithinapluripotentialstemcellorveryearlyprogenitorcellgivesrisetoleukemicstemcellsNormalstemcellLeukemicstemcell九章白血病專業(yè)知識(shí)講座第7頁九章白血病專業(yè)知識(shí)講座第8頁九章白血病專業(yè)知識(shí)講座第9頁Etiology&PathogenesisEnvironmentalfactors

AcquireddiseasesLesionstotheDNAClonalexpansion九章白血病專業(yè)知識(shí)講座第10頁Alotofenvironmentalfactorshasbeenreportedtocauseleukemia.However,onlyfourofthemarefirmlyestablishedcausalagents.Theyare:IrradiationexposureChronicbenzeneexposureChemotherapeuticagentsLeukemiavirusinfectionEnvironmentalfactorscauseleukemia九章白血病專業(yè)知識(shí)講座第11頁九章白血病專業(yè)知識(shí)講座第12頁Inheritedsyndromessuchasataxia-telangiectasia,downsyndromepredisposetosubsequentdevelopmentofleukemia.Usually,thesekindsofsyndromessharethecommonfeaturesthattheyallhavehereticdefectsintheirgenomegavebytheirparentsInheritedsyndromespredisposetoleukemia九章白血病專業(yè)知識(shí)講座第13頁AcquireddiseasepredisposetoleukemiaLeukemiamayalsodevelopfromtheprogressionofotherclonaldisordersofhematopoieticstemcells.Ploycythemiavera,idiopathicmyelofibrosis,etc九章白血病專業(yè)知識(shí)講座第14頁LeukemiaClassificationThereareatleastdozensofvarietiesofleukemia.Theyareclassifiedbyhowquicklyitprogresses.Acuteleukemiaisfast-growingandcanoverrunthebodywithinafewweeksormonths.ByContrast,chronicleukemiaisslow-growingandprogressivelyworsenoveryears九章白血病專業(yè)知識(shí)講座第15頁AcuteversuschronicleukemiaAcute:thebloodcellsofacuteleukemiaremaininanimmaturestate,sotheyreproduceandaccumulateveryrapidly.Therefore,theyneedtreatmentimmediately,otherwisethediseasemaybefatalwithinfewmonthsChronic:inChronicleukemia,thebloodcellseventuallymature,orpartiallymature.Buttheyarenot“normal”.Theyremaininthebloodmuchlongerthannormalbloodcellsandtheycannotactfunctionalcellswell九章白血病專業(yè)知識(shí)講座第16頁MyelogenousversuslymphocyticleukemiaIftheleukemiccellsarisefrommyeloidpluripotentialstemcells:myeloidleukemiaIftheleukemiccellsarisefromlymphocyticpluripotentialstemcells:lymphocyticleukemia九章白血病專業(yè)知識(shí)講座第17頁ClinicalmanifestationsLeukemichematopoiesisNormalhematopoiesismarrowfailureInfiltration九章白血病專業(yè)知識(shí)講座第18頁MarrowfailureAnemia(lossoferythocytes):fatigues,pallorweakness,reducedexercisetoleranceFeverandinfection(Poorinfectionfighters)Abnormalbleeding(lossofplatelets)九章白血病專業(yè)知識(shí)講座第19頁InfiltrationsOraltissue:swollenpainful,andbleedinggumsSplenomegalyandhepatomegalyLymphnodeenlargementBoneorjointpainCNS-headaches,seizures,weakness,blurredvisionandvomiting九章白血病專業(yè)知識(shí)講座第20頁九章白血病專業(yè)知識(shí)講座第21頁

BloodtestfindingsAnemiaisaconstantfeature.Nucleatedredcellsorimmatureredbloodcellmaybepresent.Thrombocytopeniaisnearlyalwayspresentatthetimeofdiagnosis.Thetotalleukocytecountscanbehigh,normalorlow.

Immaturehematopoieticcellsarealmostpresentintheblood九章白血病專業(yè)知識(shí)講座第22頁MarrowfindingsNormalbonemarrowAMLmarrow九章白血病專業(yè)知識(shí)講座第23頁Cytogeneticfindings九章白血病專業(yè)知識(shí)講座第24頁Diagnosis&ClassificationOthernewlydevelopedmethods九章白血病專業(yè)知識(shí)講座第25頁Morphology:thebonemarrowcellsareevaluatedaccordingtotheirsize,shape,andcontentofgranulesandthentheyareclassifiedwithrespectedtomaturityCytochemistrystaining:identificationofthechemicalcomponentsofcellsisconductedtodistinguishdifferenttypesofleukemia.Cytochemistryoftenusespecialcoloreddyes九章白血病專業(yè)知識(shí)講座第26頁AcuteleukemiaAMLALLM0:undifferentiatedAMLM1:Myeloblasticleukemia(withoutmaturation)M2:Myeloblasticleukemia(withmaturation)M3:promyelocyticleukemiaM4:MyelomonocyticleukemiaM5:MonocyticleukemiaM6:ErythroleukemiaM7:MegkaryoblasticleukemiaL1:MatureappearinglymphoblastsL2:ImmatureandvariouslyshapedlymphoblastsL3:Lymphoblastsarelargeanduniform九章白血病專業(yè)知識(shí)講座第27頁九章白血病專業(yè)知識(shí)講座第28頁P(yáng)142(CDtables)AlotofCDprovidescluesforthediagnosis九章白血病專業(yè)知識(shí)講座第29頁FlowCytometryImmunohistochemistry九章白血病專業(yè)知識(shí)講座第30頁

ImmnuophenotypingpanelusedinSt.JudeChildren’sresearchhospitalU.S.A.CD13CD33CD19CytoCD79aCD7CytoCD3AML----B-ALL----T-ALL----Byusingthismethodofanalysis,onecanmakeafirmdiagnosisin99%ofcases九章白血病專業(yè)知識(shí)講座第31頁免疫表型分型方案T細(xì)胞B細(xì)胞（4%）B細(xì)胞前體

CD7（敏感），cCD3（特異）CD19(敏感),cCD79a(特異)成熟T細(xì)胞（18%）前T細(xì)胞（6%）前B-細(xì)胞（9%）早期前-B細(xì)胞（52%）前-前-B細(xì)胞（11%）sIg,sIg九章白血病專業(yè)知識(shí)講座第32頁九章白血病專業(yè)知識(shí)講座第33頁Inserttable90%ofthecaseswithleukemiahavenon-randomizedtranslocation九章白血病專業(yè)知識(shí)講座第34頁P(yáng)118typesoftranslocationsCMLAML-M2AML-M3AML-M4AMLAML-M4E0九章白血病專業(yè)知識(shí)講座第35頁九章白血病專業(yè)知識(shí)講座第36頁九章白血病專業(yè)知識(shí)講座第37頁九章白血病專業(yè)知識(shí)講座第38頁Othernewdevelopedmethods九章白血病專業(yè)知識(shí)講座第39頁DifferentialDiagnosisPseudoleukemiaMyelodysplasticsyndromeNonleukemicpancytopenia九章白血病專業(yè)知識(shí)講座第40頁TreatmentSupportivecaresandpreparationofthepatientsAntileukemictherapyTherapyofthecentralnervoussystemStemcelltransplantation九章白血病專業(yè)知識(shí)講座第41頁AnemiaHemorrhageInfection九章白血病專業(yè)知識(shí)講座第42頁

HematologicalsupportTransfusionofPlateletsTransfusionofpackedredcellsTransfusionsofgranulocytesTransfusionsofIgG九章白血病專業(yè)知識(shí)講座第43頁

InfectioncontrolSpecialprecautions:protectiveisolationEliminationofcontaminatedfoodsOralanddigestivesystemcareBroadspectrumantibioticsGM-CSFadministration九章白血病專業(yè)知識(shí)講座第44頁AntileukemictherapyChemotherapytokillleukemiacellsusingstronganti-cancerdrugs九章白血病專業(yè)知識(shí)講座第45頁TreatmentphasesInductiontherapy:theaimistobringaboutremission,thatisleukemiccellsarenolongerfoundinthebonemarrowandtherecoveryofnormalhematopoiesisPost-remissiontherapy:toeliminateanyleukemiacellspotentialhidinginthebody九章白血病專業(yè)知識(shí)講座第46頁SpecialsubtypesAcutepromyelocyticLeukemia(APL):becauseofthesmallparticles(procoagulants)insidetheAPLcell,DICarecommonlyseeninthistypeofAML九章白血病專業(yè)知識(shí)講座第47頁NormalAPLGenesessentialfordifferentiationareshutdownbythefusionproteinsPML-RARalpha九章白血病專業(yè)知識(shí)講座第48頁Differentiationagents(all-transretinoicacid,arsenictrioxide)九章白血病專業(yè)知識(shí)講座第49頁Hyperleukecytosis:causeabatchofcomplicationsandshouldbetreatedusingleukapheresis(exchangingtransfusion)orpreinductiontreatmentpriortointensivechemotherapy九章白血病專業(yè)知識(shí)講座第50頁

Acuteleukemiaover60yearsofageislessresponsivetochemotherapyStandardtwo-drugstherapycanbeused.CR30%Intoleranttostandardtherapy.Low-doseofAra-Ccanbeusedfor14-28days九章白血病專業(yè)知識(shí)講座第51頁TherapyoftheCNSleukemiaTheCNSisafrequentsanctuarysiteforleukemiccellsbecausemostofthechemotherapydrugsarenotabletoreachtheCNS.SometypesofacuteleukemiahavetendencytobewithCNSleukemiasuchasAML(M4,M5),ALL九章白血病專業(yè)知識(shí)講座第52頁IntrathecalchemotherapyCranialirradiation九章白血病專業(yè)知識(shí)講座第53頁StemcelltransplantationsThemainpurposeofBMTandPBSCTincancertreatmentistomakeitpossibleforpatientstoreceiveveryhighdosesofchemoth