2015版临床五年大纲血液部分

一、总 四、再生性贫 六、白血 急性白血病(Acute慢性粒细胞白血病(ChronicMyelogenousLeukemia, 九、淋巴 (Hematologic 吴 刘 √112√再生性贫112112112112112112112112112（一）（二）Aftercompletingthischapter,youshouldhaveachievedthefollowingTobefamiliarwithconstructionandfunctionofhomatologicTobefamiliarwiththemainmanifestationsofhemotologicToknowtheclassificationofhemotologic theclinicalsignifecanceoflaboratorytestsforhematologicToknowthemodalitiestopreventandtreathemalogicToknowtheupdateknowledgeof（Introductionof（一）掌握内容（二）Tomastertheconceptionofanemia,importanceofetiologicaldiagnosis,anddignositicproceduresandmethods.Tomastermorphologicandpathogeneticclassification,principleofTobefamiliarwithclinical二．教学时数：2.511.5【classhour】2.5classhours；Lecture1hourPractice1.5红细胞分布宽度（RDW、红细胞参数(MCV、MCH、MCHC)的概念，及Hb、Hct、RBCvolume,MCVhemoglobin,MCH）（meancorpuscularhemoglobinconcentration,MCHC）3项红细胞指数来进行贫血分类。可以分为大细胞性、正细胞性、单红细胞生成减少：骨髓衰竭、红系祖细胞分化、无效造血、造血功能受抑、骨合成（缺铁性贫血、珠蛋白合成等、原因不明或多因素（慢性贫血,chronicdiseaseanemia,ACD）等。包括病的表现与贫血引起各系统的表现结合病理生理讲述贫血所致的各系统的症、、三、补充造血原料(铁剂、叶酸、维生素B12)，应有针对性，避免。 LecturingDefinitionofanemia,referenceofhemoglobinandredbloodcellyticparameter(MCV,MCH,MCHC,RDW).FactorsthatcaninfluencetheHblevelsuchasage, ,altitudeofinhabitancy.Anemiaisacommonsymptomcausedbyvariousdiseasesratherthanadiseaseentity.Additionalindices:Hct,MCV,MCH,MCHC,RDW.TherelationshipbetweenHb,Hct,RBC.【EtiologyandPathogenesisAnemiacausedbyimpairedproductionofBonemarrowfailure:tativedeficiencyorqualityabnormalityofhematopoieticstemcell/erythroidprogenitorcells.IneffectioveBonemarrowBonemarrowrecementbyneosticEPOAbnormalmicroenviromentofDeficiencyofhemopoieticelements,suchasiron,folateAnemiacausedbyaccelerateddestructionorconsumption(hemolyticIntrinsicdefectsinerythrocytes:redbloodcellmembranedefects,redbloodcellenzymopathies,hemoglobinopathy.Extrinsiccondition:immuneantibody,mechanical,chemical,biologicBloodMorphologicalAccordingtoMCV,MCHandMCHC,anemiacanbeclassifiedintothreegroups:microcyticanemia,normocyticanemiaandmacrocyticanemia.PathogenicAnemiacausedbyimpairedproductionoferythrocytes:bonemarrowfailure,impairederythroidprogenitorcells,ineffectivehemapoiesis,bonemarrowsuppressionorrecementbyneosm,impairedDNAsynthesis,impairedhemesynthesis,impairedglobinsynthesis,anemiaofchronicdiseases(ACD).Aanemiacausedbyaccelerateddestruction(Hemolyticanemia):Intrinsicdefectsinerythrocytes(redbloodcellmembranedefects,redbloodcellenzymopathies,impairedglobinsynthesis--thalassemias),extrinsiccondition(immuneantibody,mechanical,chemical,biologicagents.Bloodloss:acutebloodloss,chronicbloodAdvantageanddisadvantageofthesetwoclassificationsandthesignificanceintheclinicalsetting.Degreeofanemia：minor,intermediate,severe,veryClinicalmanifestations:Includingthemanifestationsoftheunderlyingdiseasesandthemanifestationsofvarioussystemsduetoanemia.Exinthesymptomsandsignsindifferentsystemsconsideringpathophysiologyandvariousfactorsaffectingthesymptoms(thespeedoftheoccurrence,thedegreeofanemia,bloodvolume,anemianatureandDiagnosticIdentifyanemiaanditsIdentifythetypeofTolookforthecauses(isespeciallyimportant,andshouldbeDiagnosticHistory:History-takingmustbeall-aroundandsystemic.Basedonacase,informationonanemia,suchasnutritionstatus,reproduction,bleeding,drugtaken,career,familyhistory;primarydiseaseshouldbecarefullysought.Theimportanceofhistory-takinginetiologicdiagnosisshouldbeemphasized.Physicalexamination(PE):systemicPE;especiallypayattentiontothepresenceofenlargedliver,spleen,lymphnodes,signsofbleeding,nailsdeformationandabnormalPeripheralbloodtest:Hb,RBC,HCT,reticulocytecount,indicesofRBC(MCV,MCH,MCHC),bloodsmearforredcellmorphologicstudyshouldbeemphasized.Bonemarrowexamination:smearandcoreSpecializedlab.testsareindicatetofurtherevaluatetheOtherteststoclarifythecausesofanemia:urinaryandfaecaltest,bloodbiochemistry,radiology,endoscopy,etc.【PrinciplesoftherapyEliminatethecausesofanemia:totreattheunderlyingdiseases,whichisthemostSymptomatictherapy:bloodtransfusionisonlyappliedto pensatedandcriticalcomdition.Bloodcomponenttransfusion.Drugs(iron,vitaminB12,folicacid,corticosteroids,androgen,etc.)shouldonlybeusedcorrectly,accordingtodifferentclinicalindications.Splenectomymaybeeffectiveincaseswithhereditaryspherocytosis,hemoglobinopathiesorautoimmunehemolyticanemia.Immunosupressivetherapyforimmune-mediatedanemia:corticosteroidsorazathioprineforAIHA;ATGorALGforasticanemia.Stemcelltransnt:indicatingforasticanemiaandthalassemiamajorandsome(IronDeficiencyAnemia,Tomasterironmetabolism,causesofirondeficiency,pathogenesisofIDA,clinicalpresentation,lab.findings.TomasterdiagnosisandtreatmentofTobefamiliarwithprevelenceand舌萎缩并可并发舌炎、口角炎。【检查治疗（C）的价值。Contentofself-【Introduction】DefinitionofirondeficiencyEpidemiologyofIDAMorphologickeypointsandironstoragechangesinIDA.【IronmetabolismBodyirondistribution.Irondailyrequirement.Theironcycleinthebody:intake,absorption,transportation,uptakebyerythroidprecursors,storage,excretion.【EtiologyandpathogenesisIncreaseddemandandinadequateironBloodIronInfluenceofirondeficiencyonironmetabolismInfluenceofirondeficiencyonInfluenceofirondeficiencyontheotherorgansandtissuesexceptSymptomsduetoOthernon-hemapoieticmanifestations:retardation,irritability,decreasedphysicalstrength,fatigue,dysphagia,pica.Physicalfindings:pallor,koilonychia,glossitis,cheilitis,InfantandchildhoodManifestationofunderliningdiseases,suchaspeptic pletebloodcountandRBCindices,marrowironstorage,serumiron,ferritin,totalironbindingcapacity,transferrinsaturation,redcellprotoporphyrin【DiagnosisanddifferencialdiagnosisDiagnosticIdentifymicrocyticEvidenceofbodyironSpecialstudiestodelineatethecausesofironRuleout:thalassemia,sideroblasticanemia,anemiaofchronicdiseases,transferrinDiagnosisbasedMorphology:microcytic,BonemarrowdepletionofstainableDecreasedserumferritinandincreasedtotalironbindingSensitivity,specificityandutilityofdifferentironTherapeutictrial：increasedreticulocyteisanearlyindicatorofirondeficiencyafterironUnderliningcausesshouldalwaysbeinvestigatedbeforetreatmentisbegun.OnceIDAhasbeenestablished,recementtherapyshouldbeinstitutedwithoutfurtherdelay.Oralironadministration:ironpreparations,dosagesideeffects,course lirontherapy:indications,preparations,calculationdosage,side【Prophylaxis】education,publichealthservicebaselinediseasescontrol【Prognosis】dependingonbaselineTomasterpathogenesis,theclinicalmanifestationandhematologicalfeatures;thediagnosticcriteriaanddifferentialdiagnosis.Tobefamiliarwithcauses,pathologicfindingsandtherapeuticToknowaboutetiologyand大数1学时，见习时数1.5学时【classhour】2.5classhoursLecture1hourPractice1.5hours.继发性：药物与化学品、物理因素、、其他。性：病因不明讲解本病的临床表现特（贫血综合征重（severeasticanemia,SAA）【检查；阵发性睡眠性血红蛋白尿（paroxysmalnocturnalhemaglobinuria,PNH急性造血功能停滞（acutearrestofhematopoiesis恶性组织细胞病(malignant病因治疗：去除引起AA非重型再障的治疗：雄的应用，中医中药治疗，造血刺激因子（G-CSFEPO免疫抑制剂的应用：重点介绍环孢素A，ALG、ATG治疗的适应症、用法及副70%～80%患者病情得到改善，预后较好。Lecturecontentsofastic【Introduction】Asticanemia（AA）outlineand【Etiology】SecondaryAA:Drug,chemicals, physicalfactors,infectionetc.PrimaryAA:causesunknown【Pathogenesis】PotentialmechanismsresponsibleforacquiredmarrowcellfailureDirecttoxicitytohematopoieticstemAdefectinthestromalmicroenvironmentofthemarrowrequiredforImpairedproductionorreleaseofessentialhematopoieticgrowthCellularorhumoralimmunesuppressiontomarrowprogenitor【Clinicalpresentions】Illustrateitsclinicalfeaturesanddifferencebetween severetypeandnon-severetype(Onset,mainsymptoms,degreeofseveritiesandcourse.)【LaboratorytestsPancytopeniawithdecreasedabsolutereticulocyteBonemarrowsmearandbiopsy:hypocellularity,markeddecreaseinmegakaryocytesandgranulocyticanderythroidcells,whilelymphocytes,smacells,andmastcellsarerelativelyincreased.Emptyfattyspacesandafewhematopoieticcells.Hematopoieticprogenitorcellculture(CFU-GM,CFU-E,Immunologicabnmalities,especiallyTcellandLaboratoryfeaturesofacuteseveretypeandchronicDiagnosisPancytopeniaanddecreasedabsolutereticulocyteUsuallynoEvidencesforshrinkofhematopoietictissue(decreasedcellularityonmarrowsmear,decreasedhemapoietictissueandincreasedfat-filledspacesonbiopsy)ExcludeotherdiseasesleadingtoDeterminationofSevereasticanemia(SAA),veryseveryasticanemia(VSAA),non-severeasticpointoutthegistsindifferentiatialAAfromthefollowingParoxysmalnocturnalMyelodyssticAleukemicAcutearrestofOtherdiseaseswithRelationshipbetweenamongAA,MDSandDiscontinueanypotentialoffendingdrugsorSupportivecare:redbloodorettransfusionifindicated,managementofHemapoieticstemcelltransCombinationherbalmedicine.【Prevention】Administratedrugcautiouslylaborprotectiontoxicexposureearly【CourseandPrognosisHighmortalityinSAA,duetosevereinfectionorintracranialhemorrhage.ImmunosuppressivetherapyandSCTdramaticallyimprove e.Treatmenteffectsonnon-SAAisabout70%-80%.1、Tomasterpathogenesisofhemolyticanemia,keypointsof intravascularandextravascularhemolysis（includeclinicalpresentationandlaboratoryfindings）2、Tobefamiliarwith:classificationanddiagnosisofhemolyticanemia.3、Toknow principleoftreatment.大数1学时，见习时数1.5学时【classhour】2.5classhoursLecture1.0hourPractice1.5hours.物理和因素：如烧伤、人工心脏瓣膜、微血管病性溶生物因素：多种其他：如PNH细胞外在因素：免疫相关（AIHAAIHA、血型不合输血反应等）微血管病性（HUS、TTP、DIC等、生物或因素、理化因素等。【检查黄疸伴贫血：无效造血（BM内、内黄疸不伴贫血：胆红素结合（Crigler-Najiar综合征、性非溶血性黄（Gilbert综合征）Contentsof【Generalconception】hemolysisandhemolytic【EtiologyandpathogenesisIntracorpuscularMemberne-cytoskeletalExtracorpuscularMechanicaldestruction:burn,heartvalveprosthesis,microangiopathicOthers:SitesofIntravascular:hemoglobinemia,Extravascular:hemolysishappenedmainlyinmononuclearphagocytesystem(MPS,mainlyinspleen) 【Classification】mainlyintroduceetiologyandpathogenesisIntracorpuscularAquried:paroxysmalnocturnalExtracorpuscularImmune-mediate:autoimmune,drugs,bloodtypeInfectionortoxic【Clinicalpresentation】Presentationisgreatlyinfluencedbywhethertheonsetisabruptorgradual,degreeandlastingduration.Acutehemolyticanemia:acuteonset,mayhavechill,fever,backpain,urticaria,dyspnea,hypotension,renalfailure(seeninABOmismatch),ormultiorganfailure.Hemolysistriad:hemoglobinemia,hemoglobinuria,hemosiderinuinChronichemolyticanemia:graduallyonset,anemia,jaundiceandsplenomegaly【Laboratorytests】evedenceofincreasedRBCdestructionanditsclinicalTestsforincreasedredcellTestsforincreasedTeststodelineatethespecifichemolyticSpecialstudiestodelineatethecausesofWhetherishemolyticIntravascularorWhichtypeofhemolyticAnemiawithincreasedreticulocyte:acutebloodloss,IDAormegaloblasticanemiaearlyresponseaftertorecementtherapy.Jaundicewithanemia:ineffectivehemopoiesis,internalJaundicewithoutanemia:Crigler-Najiarsyndrome,GilbertTreatmenttoeliminatecauseofImmunosuppressiveagents:corticosteroid,cyclosporineA,cyclophosphamide,RedcellcomponentOthertherapies:anti-CD20antibody,folateTomastertheclinicalmanifestation,featuresoflaboratorytest,diagnosticevidences,andtheprinciplesoftreatment.Tobeamiliarwiththecurrentconception,pathogenesis,classification,epidemiologyandprognosisofthisdisease.Tobefamiliarwiththecommonlyusedtreatmentprotocolsof【Lectiuringtime】6coursestotallyacuteandchronicleukemiaeach4调细胞遗传学和/WHO2008分类。OverviewofAccordingtodifferentiationandnaturalcourse,leukemiascanbedividedintotwocategories,acuteandchronicleukemia.Introduceadvanceofacuteleukemiaclassification.Emphasizetheimportanceofgeneticeventsintheclassificationandtherapyoftheacuteleukemia.BrieflyintroduceWHO2008classificationofacuteleukemia.【Incidence】Incidenceofcommontypesof【EtiologyandpathogenesisBrieflyintroducepathogenic ,radiation,chemicalmaterials,drugs,geneticfactorsandimmunedeficiencyanditsroleinleukemia.急性白血病(Acute、正常血细胞减少的临床表（贫血及白血病细胞组织浸润的各种表（各与组织浸润的症状与体征【检查】、WBC正常或低于正常，分类可见原始、幼细胞。多有不同程度的正10%AML骨髓增生低下，称之为增生低下白血病。POX、PAS和NSENaF和改变这往往是急性白血病预后的决定因素如APL的及PML/RARα融合，ALL的t(9;22)(q34;q11)及BCR/ABL融合,flt-3等CD系列，AMLALL免疫表型的根据急性白血病临床表现的特点（贫血、、及浸润）及MICM检查的3L1、L2和重点介绍WHOAMLALL淋巴细胞增高的性疾病与ALL鉴别AA控制（ALL分阶段；免疫细胞治疗；部分使用靶向治疗，例如Ph阳性ALLALL和AML.Manifestationcausedbydecreasednormalbloodcells:infection,hemorrhage,Manifestationcausedbyleukemiainfiltration:symptomandsignsdifferfrominvolvedorgansandtissues.【LaboratorytestsBloodtest:elevatedWBCwithimmaturecellpresentinmostcases.NormalordecreasedWBCcanbeseeninsomecases.AnemiaandthrombocytopeniaarecommonBonemarrowmorphologicalstudy:markedlyhypercellularitywithblastsandimmatureprecursorspredomenent.Normalerythroidprecursorandmegacaryocytesresidualminimal.HypocellularCytochemistrystain:mainlyusedforleukemictypedifferentiation,includePOX,PAS,NAP,NSE/NaF.Cytogeneticandmolecularstudies:prognosticvalueforleukemia.Suchast(15;17)(q22;q21),PML/RARαfusiongene,t(9;22)(q34;q11)andBCR/ABLfusiongene,flt-3, monusedCDprotocol,mainlyphenotypicdifferencebetweenAMLandALL.OtherAccordingtoclinicalfeatures（anemia,hemorrhage,infection,infiltration）,laboratorytests(includebloodandmarrowmorphology,cytochemistrystaining,immunophenotype,cytogenetics),aacuteleukemiadiagnosiscanbemade(includeAMLandALL)Classification:canbeclassifiedinanumberofways.ThemostadvanceisWHOclassification.FABclassificationistheprincipalofallotherclassification.WHOclassificationbasedonMICC(morphology,immunophenotype,clinicalfeatures,cytogeneticandmolecularstudy).BrieflyintroduceWHOclassification.FABclassification:French-American-British(FAB)systemreliesonmorphology.AMLsubclassifiedasM1,M2,M3,M4,M5,M6andM7sbutypes.ALLsubclassifiedasL1,L2andL3subtypes.MyelodyssiaLeukemoidInfectiousdisorderswithHypocellularleukemiaversusasticRecoveryof【Treatment】Individualizedstratified（basedonpatientage,leukemiatype,cytogeneticfeatures）andRiskstratifiedtreatment.SupportivemedicalBloodcomponentsInfectionprevention(protectingenvironment,antibiotics,antifungalManagementNursing,nutritionandpsychologicalUricacidnephtopathypreventionandHyperleukocytosis:intracranialhemorrhageorpulmonaryinsufficiencyisthemostseriouscomplicationsofhyperleukocytosis.Promptlytherapyisimportant.Therapeuticprinciples:early,combination,sufficientdosage,intermittence,extramedullaryleukemiaprophylaxis,individualization,treatmentphases(inductionandpost-remissionCommonlyuseddrugsandchemotheraputicregimensinALLandANLL,regimensofpostremissionmaintenanceanditscourse.Centralnervoussystemleukemia leukemiaprophylaxisandStemcelltransnt:Allogeneicstemcellstransntationandautologousstemcellstransntation,eachadvantages,disadvantagesandeffectiveness.【Prognosis】naturalhistoryisabout3months.Survivalcloselyrelatedtopatientleukemiatype,especilly,cytogeneticandmolecular慢性粒细胞白血病(ChronicMyelogenousLeukemia,CML是一种造血干细胞恶性克隆性疾病。Ph、BCR/ABL融合是细胞遗传CMLCML临床过程分为三期，即慢性期、加速期及急变期。各期临床表现不一，分别加以血象：慢性期外周血WBC明显增高，可达（100～300）×109/L，中性中幼、晚幼鉴别诊断（1）与ph阳性的ALL鉴别；(2)类白血病反应；(3)骨髓纤维Chronicmyelogenousleukemia(CML)isapluripotentialstemcelldiseasecharacterizedbyanemia,extremebloodgranulocytosisandgranulocyticimmaturity,basophilia,oftenthrombocytosis,andsplenomegaly.Ph1chromosomeorBCR-ABLfusiongeneisthehallmarkpathogenesisofCML.Clinicalfeatures:usual,unusualandoccasionalsymptoms.SomepatientsarediagnosedbyThreephasesinCMLclinicalcourse：Chronicchronic,Acceleratedaccelerated,Blastphase,theirclinicalpresentationsvariable.Chronicphasecanbeasymptomaticorsymptomsduetoanemiaandsplenomegaly,fatigue,weightloss,malaise,easysatiety,andleftupperquadrantfullnessorpain.SpenomegalyisthemostconsisitentphysicalsigninCML.【LaboratorytestsBloodtest:ElevatedWBCs,withincreasesinbothimmatureandmaturegranulocytes.Decreasedactivityofneutrophilealkalinephosphase(NAP).Basophilsandeosinophilsarecommonlyincreased.Bonemarrow:markedlyhypercellularity,withincreasedmyeloid/erythroidratio.differentialcount.Myelofibrosiscanbefoundinsomeofthepatients.Chromosomeandmolecularfindings:Ph1chromosomeorBCR/ABLfusiongene.isthehallmarkofCML.Additionalchromosomalchangespresentinadvancedphases.Chemicalabnormalities:uricacid,LDHlevel.SerumVitaminB12-BindingproteinsandVitminB12.【DiagnosisanddifferentialdiagnosisDiagnosis:ThediagnosisofCMLismade,basedonthecharacteristicgranulocytosis,whitecelldifferentialcount,increasedabsolutebasophilcount,decreasedNAP,andsplenomegalycoupledwiththepresenceofthePhchromosomeoraBC/ABL.fusionLeukemoidBonemarrow【Treatment】ThegoaloftherapyinCMListoachieveprolonged,durable,nonneostic,nonclonalhematopoiesis.Completehematologicresponse(CHR),completecytogeneticresponse(CCyR),majormolecularresponse(MMR),completemolecularresponse(CMR).Chemotherapy:hydroxyureaorbusulfananditseffectandTyrosinekinaseinhibitor:imatinibandothersignaltransductioninhibitor,mechanism,dosage,effects,sideeffects,responsedefine, Hematopoieticstemcelltransnt:indication,effects,riskadjudgeOthers:irradiation,leucopheresis,Treatmentofblast【Prognosis】survivalprognostic慢性淋巴细胞白血病(ChronicLymphocyticLeukemia,多在中老年患病多于女性在WHO分类中CLL与SL（smallcelllymphoma,SCL）在同一条目下描述均属于淋巴细胞低度恶性克隆性疾病肿瘤细胞长使其在体内蓄积。浸润骨髓、淋等，最终导致造血功能衰竭。【检查淋活检：小淋巴浸润B细胞标记。CD19、CD20、CD5阳性，CD23阳性，轻链限 、q+常见并发症的治疗：控制(抗生素、静脉丙种球蛋白的使用)，免疫性血小板减【Overview】Chroniclymphocyticleukemia(CLL)predomenentinelderlypatients.Maleisslightlymorethanfemale.CLLisaneosmcharacterizedbyaccumulationofmonoclonallymphocytesofB-cellorigin.Thecellsaccumulateinthebonemarrowlymphnodes,liverspleen,andoccasionallyotherorgans.TheWHOclassificationdescribedCLL/SLLatthesameitem.MostpatientswithCLLareasymptomaticatearlyNonspecificsymptoms(Bsymptoms):fatigue,lethargy,lossofappetite,weightloss,andreducedexercisetolerance,recurrentinfections,autoimmunehemolyticanemia,andimmunethrombocytopenia.【LaboretorytestsPeripheralblood:lymphocytosis,anemiaor/andBonemarrow:theratioofmature-likelymphocytemorethanLymphnodebiopsy:iseffacedbyadiffuseinfiltrationofsmallTheimmunophenotype:typicallyareCD5+,CD10–,CD19+,CD20(dull),CD23+,CD103–,surfacelightchainrestrictuion.Chromosomeabnormality:halfpatientsarefoundtohaveclonalchromosomalabnormalities.Commonlyfinding:del13q14-23.1,trisomy12,del11q22.3-q23.1,del6q21-q23,deletionsat17p13.1and14qabnormalities.【Diagnosis】Accordingtobloodpicture（absolutelymphocytecounts5×109/L）andmarrowsmearandtheimmunophenotypeofleukemiccells.Excludeotherlymphocytosis【Clinicalstaging】IntroductionofBinetstaging：AB【Treatment】StratifyaccordingtoclinicalconditionandChemotherapy:Indication,dosage,courseandeffectivenessofchlorambucil,prednisone,cyclophosphamide,andfludarabine.HematopoietictemcelltransIrriationSupportivecare:Managementofinfection(antibiotics,humanIgGinfusion)hemolyticanemiaandimmune【Prognosis】Naturalhistory,survivalaftertreatment,andcausesofmortality(hemorrhage,(MyelodyssticSyndrome,掌握MDS临床表现、检查特点、诊断及鉴别诊断MDS的WHOMDSTomaster:MDSclinicalmanifestation,laboratoryfeatures,diagnosisanddifferentialTobefamiliarwithWHOTounderstandhowtotreatandhowabouttheir大数1学时，见习时数1学时【classhour】2classhoursLecture1hourPractice1hour.些的突变等。FAB、WHO分型的特点、差异；重点介绍WHO【和骨髓病理学:大多增生活跃，个别增生低下，可见ALIP：5q-血的依据、细胞遗传学异常、ALIP(abnormallocalizationofimmatureprecursor)等。有造血(dyssia)的其他疾患：骨髓增生症、骨髓造血组织肿瘤及其他非nocturnalhemoglobinuria,PNH）等；表观修饰治疗抗血管新生治疗：胺等化疗：RAEB的MDS患者小于50岁，可采用与AML相同的标准化疗。较大者可用小剂量的Ara-C治疗。MDSLecture【Overview】conceptionofMDSgeneralaspects,evolutionof【EtiologyandpathagenesisMDSisaclonaldisorderwithanacquiredsomaticmutationthataffectsanearlyhematopoieticprogenitorandgivesrisetoclonallyderivedneutrophils,redcells,andThedyssiarelatedtoabnormalityofproliferation,apoptosisand【Classification】IntroduceFABandWHOclassificationmainlyonWHOMDSisadiseaseoftheelderly.Patientsusuallypresentwithanemia,infectionpredispositionandhemorrhage.ClinicaldiversityrelatedtodifferenttypesofMDS.【LaboratorystudiesBlood:pancytopenia,oriso-/bi-Bonemarrow:hypercellularityorhypocellularity.Evidenceofdysmyelopoiesis.Onmarrowbiopsy,ALIP,micromegakaryocytecanbeseen.Cytogenetics【DiagnosisanddifferentialDiagnosis:iso-/bi-/pancytopenia,dyssiticfeaturesofmorphologyinbloodormarrow,cytogeneticabnormalities,ALIP.DifferentialSomediseaseswithdyssia,suchasmyelopreliferativedisorsers,hemotologicneosmsandnon-hematologicneosms.Pancytopenia:asticanemia,MegaloblasticHemolyticSupportivecare:bloodcomponenttransfusion,preventionandtreatmentofinfection,bleedingcontrol,VitaminB12/B6,folicacid.:HematopoieticStemCellTransEpigeneticAntiangiogenesisagents:thalidomide,Conventionalchemotherapy:standarddosechemotherapyforagelessthan50,lowdoseAra-Cforelderly.Others:thereisnoclearrole,despiteanecdotalreportsofCorticosteroidsorimmunosuppressiveThreekindsof e:transformtoovertleukemia,marrowfailure,succumbtootherdiseasesunrelatedtotheirMDS.（Myeloproliferativeneosm,了解真性红细胞增多症（polycythemiavera,PV、性血小板增多症(essentialthrombocytosis,ET)、性骨髓纤维化(primarymyelofibrosis,PMF)的临床表现、Master:definitionandfeaturesofFamillarwith:MPNanddifferentiationwithsecondarymarrowproliferation(secondaryerythrocytosis,thrombocytosis,andmarrowfibrosis).Knowing:clinicalmanifestation,lab.tests,treatmentofPV,ET,PMF.TodifferentiatePV,ET,andPMF.大数1学时，见习时数1学时【classhour】2classhoursLecture1hourPractice1hour.MPN是一组造血干细胞克隆增殖的肿瘤性疾病，表现为一系或多系分化相对成骨发性血小板增多症、性骨髓纤维化和不能分类的MPN。【MPN髓外化生(extramedullarymetasia)。2008WHO分类进展，MPNMPDMPN疾病MPN分类。学异常，JAK2PVMPN中的价值。2008WHO诊断标准的变迁，PV的诊断标准、ET的诊断标准、PMF血后、慢性或急染、肿瘤性疾病等。LectureMPNsareclonalhematopoieticstemcelldisorderscharicterisedbyproliferationofoneormoreofthemyeloidlineages.Theproliferationisassociatedwithrelativelynormalmaturation.MPNsincludepolycythaemiavera(PV),CML,chroniceosinophilicsyndrome,ET,PMFandMPN,unclassifiable.IntroduceWHO2008classification.Towardsgeneticclassificationanddiagnosisofmyeloid【CommoncharacteristicsofMPN】Shareacommonstemcell-derivedclonalheritagetoabnormalsignaltransduction.Phenotypediverse,oneormoreofthemyeloid,granulocytic,erythroidandmegakaryocyticlineagesproliferation.ClassicMPNclassification.Overlapoftheclinical,laboratoryfindings.Myeloidmetasia.【Clinicalmanifestation】reflectthepatternofabnormalitiesincellproductionandClinicalfeaturesofeachBloodandmarrow【DiagnosisWHO2008diagnositcriteriabrieflyintroducethediagnosticcriteriaforPVET,PV：differentialwithsencondaryET:withsecondarythrombocytosis(splenectomy,hemolyticanemia,acutebloodloss,chronicoracuteinfectiousdiseases,malignancy)PMF:withsecondarymarrowfibrosis(carcinoma,Symptomatictreatment:erythropheresisorphlebotomy,Marrowsuppressivetherapy:hydroxyuria,alkyletingSignaltransductioninhibitorandtargettherapy:JAK2Hematopoieticstemcelltrans【Prognosis】endstagemaybemarrowfailureortransformtoacute(Lymphoma:Hodgkinlymphoma,HL;nonHodgkinlymphoma,【PurposeandandrequirementTomaster:diagnosticandtherapeuticprinciples;clinicalmamifestation;diagnosticcriteria,andclinicalstaging.Tobefamiliarwith大数1学时，见习时数1.5学时【classhour】2.5classhoursLecture1hourPractice1.5hour.EB与Burkitt淋巴瘤及HL的关系HLNHLWHO2008年淋巴及其他表现。比较HL与NHL症状的不同。【和B超检查和核素显象可发现体检遗漏淋及深部（PET/CE淋活检与印TCR和B-细胞重链重排检死淋炎、恶性组织细胞增多症相鉴别。治疗原则，强调高、中恶性和低恶性NHL国际预后指数（internationalprognosisindex,IPI）介绍（、临床分期、体能、LDHLecture【Overview】Generalaspectsand【EtiologyandpathogenesisEBVanditsroleinBurkitt,Hodgkin andtheirrolesinHelicobacterpylorianditsroleinMALTImmunosuppressionanditsroleinBrieflyintroducetheevolutionofclassification,mainlyrecentrevisedWHO2008lymphomapathologyandPainlessandprogressiveManifestationofextranodalSystemicMainlydifferentmanifestationbetweenHodgkinandNon-Hodgkin【EvaluationtestsBloodandbonemarrowSuperficiallymphnodescanbepalpableonphysicalUltrosonography,CT,PET/CE,detectingenlargeddeeplymphnodes,organPathologicLymphnodesbiopsyandtouchLymphocyteCD:todefinecelloriginandderivedfromprecursorormaturecells.ChromosomalTCRorIgHExploratory【Diagnosis,differentialdiagnosisandstagingDiagnosis:accordingtoclinicalmanifestation,pathologicevidence,immunophenotype,lymphomadiagnosiscanbemade.Stagingbasedonanatomicsitesofinvolveddisease.Differentialdiagnosis:tuberculosis,septicemia,connectivetissuediseases,histiocyticnecrotizinglymphadenopathy.Staging:stagingforHodgkinandnon-Hodgkin【Treatment】stratifiedtherapyaccordingtoclinicalconditions,degreeofmalignancy,Radiationtherapy:indication,modeandChemotherapy:indication,commonlyuseddrug,combinationtherapy,Hematopoietcstemcelltransantibodies(anti-CD20Ab)【Prognosis】internationalprognosisindex,(IntroductiontoHemorrhagic掌握性疾病的分类、特点，发现及诊断Tomasterthenormalmechanismofhemostasis,coagulationandTomastertheclassificationsofhemorrhagicdisorders,thebleedingfeaturesofeachclassification,laboratoryfindingsanddiagnosis.Tobefamiliarwithprinciplesof【classhour】2.5classhoursLecture1hourPractice1.5hours.】【概述性疾病的定义。强调自发或后不止或过多是性疾病的】ⅢCProtrinC、ProteinS及凝血酶调节蛋白（thrombomodulin,TM）组FVFⅧ。inhibito,TFPI【性疾病分类凝血功能【检查筛选实验：束臂试验、血小板计数、器时间(TBT)、血块退缩试验、凝血（APTT确诊试验：①血管异常检查；②血小板异常检查；③凝血异常检查：PT、APTT纠消除病因、避免损伤、禁用影响止血的药物，预防CAI课件课堂双语讲授，临床实习时可将适当病例进行分析、讨Lecture【IntroductionConception,featuresandclassificationsofhemorrhagicdisorders.Emphasizespontaneousbleedingorexcessivehemorrhageisthekeypointsofhemorrhagicdisorders.【Normalhemostasisandcoagulation】the3pointsofConstructionandfunctionofAdhesion,aggregationandreleasereactionofetsanditsrolesinCoagulationcascade:eachcoagulationfactors,intrinsic,extrinsicandcommonpathways;andthedynamicbalancebetweenanticoagulationandfibrinolysis.Emphasizeinvivo,onthatintrinsicandextrinsicarecloselyinterlinkedandextrinsicpasswayisfirst【Mechanismofanticoagulationandfibrinolysis】invivo,coagulationandmaintainlydynamicProteinCsystem:composedofPC,PSandthrombomodulinTiss