白血病英文課件

第六篇血液系統(tǒng)疾病

第九章白血病(Leukemia)周劍峰學(xué)時數(shù)：3學(xué)時講授目的和要求1.掌握急、慢性白血病的臨床表現(xiàn)，實驗室檢查及診斷標(biāo)準(zhǔn)，治療原則2.熟悉急性白血病FAB分型，聯(lián)合化療的原則，完全緩解的概念講授主要內(nèi)容概述病因和發(fā)病機制臨床表現(xiàn)實驗室檢查診斷標(biāo)準(zhǔn)鑒別診斷治療Erythrocytes:transportoxygenNeutrophilBasophilEosinophilMonocytes/MacrophageDefenseagainstinfectionPlatelets:MediatebloodclottingT-lymphocytes:antigenpresentingB-lymphocytesPlasmacell:SourceofantibodiesPluripotentialstemcellsMyeloidstemcellsLymphoidstemcellsUnipotentialprogenitorcellsImmaturehematopoieticcellsmaturehematopoieticcellsHematopoiesiscomposesoftheoptionsofcommitmenttodifferentlineagesandtheprogressivestagesofmaturationatwhichpartialorcompletearrestcanoccur,resultsinthewidearrayofmalignantdisease-LeukemiaStemcellProgenitorcellImmaturecellMaturecellAccumulationofmutationsofDNAwithinapluripotentialstemcellorveryearlyprogenitorcellgivesrisetoleukemicstemcellsNormalstemcellLeukemicstemcellEtiology&PathogenesisEnvironmentalfactors

AcquireddiseasesLesionstotheDNAClonalexpansionAlotofenvironmentalfactorshasbeenreportedtocauseleukemia.However,onlyfourofthemarefirmlyestablishedcausalagents.Theyare:IrradiationexposureChronicbenzeneexposureChemotherapeuticagentsLeukemiavirusinfectionEnvironmentalfactorscauseleukemiaInheritedsyndromessuchasataxia-telangiectasia,downsyndromepredisposetosubsequentdevelopmentofleukemia.Usually,thesekindsofsyndromessharethecommonfeaturesthattheyallhavehereticdefectsintheirgenomegavebytheirparentsInheritedsyndromespredisposetoleukemiaAcquireddiseasepredisposetoleukemiaLeukemiamayalsodevelopfromtheprogressionofotherclonaldisordersofhematopoieticstemcells.Ploycythemiavera,idiopathicmyelofibrosis,etcLeukemiaClassificationThereareatleastdozensofvarietiesofleukemia.Theyareclassifiedbyhowquicklyitprogresses.Acuteleukemiaisfast-growingandcanoverrunthebodywithinafewweeksormonths.ByContrast,chronicleukemiaisslow-growingandprogressivelyworsenoveryearsAcuteversuschronicleukemiaAcute:thebloodcellsofacuteleukemiaremaininanimmaturestate,sotheyreproduceandaccumulateveryrapidly.Therefore,theyneedtreatmentimmediately,otherwisethediseasemaybefatalwithinfewmonthsChronic:inChronicleukemia,thebloodcellseventuallymature,orpartiallymature.Buttheyarenot“normal”.TheyremaininthebloodmuchlongerthannormalbloodcellsandtheycannotactfunctionalcellswellMyelogenousversuslymphocyticleukemiaIftheleukemiccellsarisefrommyeloidpluripotentialstemcells:myeloidleukemiaIftheleukemiccellsarisefromlymphocyticpluripotentialstemcells:lymphocyticleukemiaClinicalmanifestationsLeukemichematopoiesisNormalhematopoiesismarrowfailureInfiltrationMarrowfailureAnemia(lossoferythocytes):fatigues,pallorweakness,reducedexercisetoleranceFeverandinfection(Poorinfectionfighters)Abnormalbleeding(lossofplatelets)InfiltrationsOraltissue:swollenpainful,andbleedinggumsSplenomegalyandhepatomegalyLymphnodeenlargementBoneorjointpainCNS-headaches,seizures,weakness,blurredvisionandvomiting

BloodtestfindingsAnemiaisaconstantfeature.Nucleatedredcellsorimmatureredbloodcellmaybepresent.Thrombocytopeniaisnearlyalwayspresentatthetimeofdiagnosis.Thetotalleukocytecountscanbehigh,normalorlow.

ImmaturehematopoieticcellsarealmostpresentinthebloodMarrowfindingsNormalbonemarrowAMLmarrowCytogeneticfindingsDiagnosis&ClassificationOthernewlydevelopedmethodsMorphology:thebonemarrowcellsareevaluatedaccordingtotheirsize,shape,andcontentofgranulesandthentheyareclassifiedwithrespectedtomaturity.Cytochemistrystaining:identificationofthechemicalcomponentsofcellsisconductedtodistinguishdifferenttypesofleukemia.Cytochemistryoftenusespecialcoloreddyes.AcuteleukemiaAMLALLM0:undifferentiatedAMLM1:Myeloblasticleukemia(withoutmaturation)M2:Myeloblasticleukemia(withmaturation)M3:promyelocyticleukemiaM4:MyelomonocyticleukemiaM5:MonocyticleukemiaM6:ErythroleukemiaM7:MegkaryoblasticleukemiaL1:MatureappearinglymphoblastsL2:ImmatureandvariouslyshapedlymphoblastsL3:LymphoblastsarelargeanduniformP142(CDtables)AlotofCDprovidescluesforthediagnosisFlowCytometryImmunohistochemistry

ImmnuophenotypingpanelusedinSt.JudeChildren’sresearchhospitalU.S.A.CD13CD33CD19CytoCD79aCD7CytoCD3AML----B-ALL----T-ALL----Byusingthismethodofanalysis,onecanmakeafirmdiagnosisin99%ofcases免疫表型分型方案T細胞B細胞（4%）B細胞前體

CD7（敏感），cCD3（特異）CD19(敏感),cCD79a(特異)成熟T細胞（18%）前T細胞（6%）前B-細胞（9%）早期前-B細胞（52%）前-前-B細胞（11%）sIg,sIgInserttable90%ofthecaseswithleukemiahavenon-randomizedtranslocation