白血病――血液系统疾病――内科学

白血病(Leukemia)第六章血液系统疾病讲授目的和要求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PluripotentialstemcellsMyeloidstemcellsLymphoidstemcellsUnipotentialprogenitorcellsImmaturehematopoieticcellsmaturehematopoieticcellsProgenitorcellMaturecellEtiology&PathogenesisEnvironmentalfactorsAcquireddiseasesLesionstotheDNAClonalexpansionCytogeneticfindingsDiagnosis&ClassificationOthernewlydevelopedmethodsFlowCytometryImmunohistochemistry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P118typesoftranslocationsCMLAML-M2AML-M3AML-M4AMLAML-M4E0OthernewdevelopedmethodsDifferentialDiagnosisPseudoleukemiaMyelodysplasticsyndromeNonleukemicpancytopeniaTreatment1.Supportivecaresandpreparationofthepatients2.Antileukemictherapy3.Therapyofthecentralnervoussystem4.StemcelltransplantationAnemiaHemorrhageInfectionHematologicalsupportTransfusionofPlateletsTransfusionofpackedredcellsTransfusionsofgranulocytesTransfusionsofIgGInfectioncontrolSpecialprecautions:protectiveisolationEliminationofcontaminatedfoodsOralanddigestivesystemcareBroadspectrumantibioticsGM-CSFadministrationAntileukemictherapyChemotherapytokillleukemiacellsusingstronganti-cancerdrugsTreatmentphasesInductiontherapy:theaimistobringaboutremission,thatisleukemiccellsarenolongerfoundinthebonemarrowandtherecoveryofnormalhematopoiesisPost-remissiontherapy:toeliminateanyleukemiacellspotentialhidinginthebodySpecialsubtypesAcutepromyelocyticLeukemia(APL):becauseofthesmallparticles(procoagulants)insidetheAPLcell,DICarecommonlyseeninthistypeofAMLNormalAPLGenesessentialfordifferentiationareshutdownbythefusionproteinsPML-RARalphaDifferentiationagents(all-transretinoicacid,arsenictrioxide)IntrathecalchemotherapyCranialirradiation复习思考题1.慢性粒细胞白血病与类白血病反应的鉴别要点？2.白血病完全缓解的标准？为何要进行巩固、强化化疗？3.骨髓增生异常综合征的分型、临床表现、与急性白血病的关系？谢谢！