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1、第六篇血液系统疾病 第九章 白血病 Leukemia 周剑峰 学时数 3学时 讲授目的和要求 1 掌握急 慢性白血病的临床表现 实验室检查及诊断标准 治疗原则2 熟悉急性白血病FAB分型 联合化疗的原则 完全缓解的概念 讲授主要内容 概述病因和发病机制临床表现实验室检查诊断标准鉴别诊断治疗 Erythrocytes transportoxygen NeutrophilBasophilEosinophilMonocytes Macrophage Defenseagainstinfection Platelets Mediatebloodclotting T lymphocytes antigen
2、presenting B lymphocytesPlasmacell Sourceofantibodies Pluripotentialstemcells Myeloidstemcells Lymphoidstemcells Unipotentialprogenitorcells Immaturehematopoieticcells maturehematopoieticcells Hematopoiesiscomposesoftheoptionsofcommitmenttodifferentlineagesandtheprogressivestagesofmaturationatwhichp
3、artialorcompletearrestcanoccur resultsinthewidearrayofmalignantdisease Leukemia Stemcell Progenitorcell Immaturecell Maturecell AccumulationofmutationsofDNAwithinapluripotentialstemcellorveryearlyprogenitorcellgivesrisetoleukemicstemcells Normalstemcell Leukemicstemcell Etiology Pathogenesis Environ
4、mentalfactorsAcquireddiseases LesionstotheDNA Clonalexpansion Alotofenvironmentalfactorshasbeenreportedtocauseleukemia However onlyfourofthemarefirmlyestablishedcausalagents Theyare IrradiationexposureChronicbenzeneexposureChemotherapeuticagentsLeukemiavirusinfection Environmentalfactorscauseleukemi
5、a Inheritedsyndromessuchasataxia telangiectasia downsyndromepredisposetosubsequentdevelopmentofleukemia Usually thesekindsofsyndromessharethecommonfeaturesthattheyallhavehereticdefectsintheirgenomegavebytheirparents Inheritedsyndromespredisposetoleukemia Acquireddiseasepredisposetoleukemia Leukemiam
6、ayalsodevelopfromtheprogressionofotherclonaldisordersofhematopoieticstemcells Ploycythemiavera idiopathicmyelofibrosis etc LeukemiaClassification Thereareatleastdozensofvarietiesofleukemia Theyareclassifiedbyhowquicklyitprogresses Acuteleukemiaisfast growingandcanoverrunthebodywithinafewweeksormonth
7、s ByContrast chronicleukemiaisslow growingandprogressivelyworsenoveryears Acuteversuschronicleukemia Acute thebloodcellsofacuteleukemiaremaininanimmaturestate sotheyreproduceandaccumulateveryrapidly Therefore theyneedtreatmentimmediately otherwisethediseasemaybefatalwithinfewmonthsChronic inChronicl
8、eukemia thebloodcellseventuallymature orpartiallymature Buttheyarenot normal Theyremaininthebloodmuchlongerthannormalbloodcellsandtheycannotactfunctionalcellswell Myelogenousversuslymphocyticleukemia Iftheleukemiccellsarisefrommyeloidpluripotentialstemcells myeloidleukemia Iftheleukemiccellsarisefro
9、mlymphocyticpluripotentialstemcells lymphocyticleukemia Clinicalmanifestations Leukemichematopoiesis Normalhematopoiesis marrowfailure Infiltration Marrowfailure Anemia lossoferythocytes fatigues pallorweakness reducedexercisetoleranceFeverandinfection Poorinfectionfighters Abnormalbleeding lossofpl
10、atelets Infiltrations Oraltissue swollenpainful andbleedinggumsSplenomegalyandhepatomegalyLymphnodeenlargementBoneorjointpainCNS headaches seizures weakness blurredvisionandvomiting BloodtestfindingsAnemiaisaconstantfeature Nucleatedredcellsorimmatureredbloodcellmaybepresent Thrombocytopeniaisnearly
11、alwayspresentatthetimeofdiagnosis Thetotalleukocytecountscanbehigh normalorlow Immaturehematopoieticcellsarealmostpresentintheblood Marrowfindings Normalbonemarrow AMLmarrow Cytogeneticfindings Diagnosis Classification Othernewlydevelopedmethods Morphology thebonemarrowcellsareevaluatedaccordingtoth
12、eirsize shape andcontentofgranulesandthentheyareclassifiedwithrespectedtomaturityCytochemistrystaining identificationofthechemicalcomponentsofcellsisconductedtodistinguishdifferenttypesofleukemia Cytochemistryoftenusespecialcoloreddyes Acuteleukemia AML ALL M0 undifferentiatedAMLM1 Myeloblasticleuke
13、mia withoutmaturation M2 Myeloblasticleukemia withmaturation M3 promyelocyticleukemiaM4 MyelomonocyticleukemiaM5 MonocyticleukemiaM6 ErythroleukemiaM7 MegkaryoblasticleukemiaL1 MatureappearinglymphoblastsL2 ImmatureandvariouslyshapedlymphoblastsL3 Lymphoblastsarelargeanduniform P142 CDtables AlotofC
14、Dprovidescluesforthediagnosis FlowCytometry Immunohistochemistry ImmnuophenotypingpanelusedinSt JudeChildren sresearchhospitalU S A CD13CD33CD19CytoCD79aCD7CytoCD3AML B ALL T ALL Byusingthismethodofanalysis onecanmakeafirmdiagnosisin99 ofcases 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
15、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 Inserttable 90 ofthecaseswithleukemiahavenon randomizedtranslocation P118typesoftranslocations CML AML M2 AML M3 AML M4 AML AML M4E0 Othernewdevelopedmethods DifferentialDiagnosis Pseudoleukemia Myelodysplasticsyndrome Nonleukemicpancytopenia T
16、reatment SupportivecaresandpreparationofthepatientsAntileukemictherapyTherapyofthecentralnervoussystemStemcelltransplantation AnemiaHemorrhageInfection HematologicalsupportTransfusionofPlateletsTransfusionofpackedredcellsTransfusionsofgranulocytesTransfusionsofIgG InfectioncontrolSpecialprecautions protectiveisolation Eliminationofcontaminatedfoods Oralanddigestivesystemcare Broadspectrumantibiotics GM CSFadministration Antileukemictherapy Chemotherapytokillleukemiacellsusingstronganti cancerdru
