教学贫血全面版课件

教学教学贫血血第1页/共43页教学贫血第1页/共43页1DefinitionA reduction below normal in the concentration of hemoglobin,the mass of red blood cells and/or the hematocrit in the blood.In men Hb 120g/L RBC 4.51012/L HCT 0.42 In women Hb 110g/L RBC4.0 1012/L HCT0.37 第2页/共43页DefinitionAreductionbelowno2Factors influencing Hb concentrationSexAgeAltitudeAlterations in plasma volume第3页/共43页FactorsinfluencingHbconcent3Classification Morphologic classificationTypeMCV(um)MCH(PG)MCHC(%)DiagnosisMacrocytic1003232-35Megaloblastic anemiaNormocytic80-10026-3232-35AA,HA,Acute posthemorragic anemiaMicrocytic802632IDA,Sideroblastic anemiaThalassemias第4页/共43页ClassificationMorphologic4Kinetic classification(Classified according to etiology and pathogenesis)一、一、Decreased erythropoiesisDecreased erythropoiesis 1Proliferation and differentiation abnormalities of SCHSC:AA,Fanconisanemia,MDSErythrocyticprogenitors:Pureredcellaplasia,Anemias caused by kidney failureandendocrinedisorders第5页/共43页Kineticclassification(Classi52Marrow infiltration leukemiascarcinomamultiple myeloma myelofibrosisMalignant histocytosis第6页/共43页Marrowinfiltrationleukemi63Production and maturation blockages of differentiating cells DNAsynthesisblockage:VitB12,falicaciddifficiencies,嘌呤和嘧啶嘌呤和嘧啶metabolicdefectsMegaloblasticanemiaHbsynthesisblockage:Hemeproductiondefect:IDAHeptoglobinproductiondefect:Thalassemias第7页/共43页Productionandmaturationbloc7ClinicalmanifestationsGeneralmanifestations:(Nonspecificandreflecttissuehypoxia)Tiredness,fatiguethemostcommon,theearliestsymptomPallorofskinandmucosasharedchracteristicCardiovascularsystems:Palpitationsanddyspneaonexertion,breathlessnesscommonsymptomsValvulaemurmursHb3mAnemiccardicdiseaseHereditaryspherocytosisAIHAHypersplenism由于胸腺瘤(Thymoma)引起的纯红AA，切除胸腺可使部分患者病情缓解。Theagedpatientsorpatientswithvascularorcardiacdiseasesmaynotstandtomilderanemia.1）Endogenous(intra-erythrocytedefects)1.Androgens:maypromatereythropoiesis:三、LaboratoryfindingsMechanical:marchhemoglobinuria,cardiacvalvesprostheses,microangiopathichemolyticdisorders.ThalassemiasDuetochemical,physicalormicrorganisms:Chemicaltoxin-anddrug-inducedhemolysis,largescaleburnedpatient(severeburnedpatient)Infection-induced,benzene,radiationChronicITP5)ImmunologictestsProliferationanddifferentiationClassificationMegaloblasticanemia,IDAHypoplasticanemias(增生减低性贫血):AAProliferationanddifferentiation2)TestsforhemolyticanemiasuchasCoombstest4 Unknown reason or several mechanismsSideroblasticanemiaChronicdiseaseanemias(chronicinflummation,infections,尿毒症尿毒症,Hepaticdisorders,neoplasm,connectivetissuedisease,endocrinedisorders)第8页/共43页Clinicalmanifestations4Unk8二、二、Accelerateddestructionofredcells1）Endogenous(intra-erythrocytedefects)1.Membrane defects of erythrocytes:Hereditary:Hereditaryspherocytosis(HS)Hereditasyelliplocytosis(HE)Acquired:PNH2.Enzyme defectsGlucose-6-PhosphateDehydrogenase(G6PD)deficiencyPyruvatekinasedeficiency3.Abnormal haptogllbin synthesis:Sicklecellanemia,HemoglobinopathiesThalassemias第9页/共43页二、Accelerateddestructionofr92)Extragenous1.Immune HA:AIHA,neonatalHA,mismatchedtransfusion,drug-inducedHA2.Mechanical:marchhemoglobinuria,cardiacvalvesprostheses,microangiopathichemolyticdisorders.3.Due to chemical,physical or microrganisms:Chemicaltoxin-anddrug-inducedhemolysis,largescaleburnedpatient(severeburnedpatient)Infection-induced,benzene,radiationmicrorganisms:Malaria,virus,etc.4.Increased damage of monocyte-macrocyte system:Hypersplenism第10页/共43页2)ExtragenousImmuneHA:AIHA,10三三.BloodlossAcuteposthemorrageanemiaChronicbleedingIDA第11页/共43页三.BloodlossAcutepos11ClassifiedaccordindtotheproliferativesituationsofBMHyperplastic anemias(增生性贫血):Hemolytic anemiaAnemia caused by blood lossMegaloblastic anemia,IDA Hypoplastic anemias(增生减低性贫血):AA第12页/共43页Classifiedaccordindtothepr12ClinicalmanifestationsFactors influencing symptoms:SeverityofanemiaHb8090g/LsymptomsRapidityofanemiaAbruptlossof20%ofthecirculatingbloodvolumemarkedpallor,posturalhypotensionandtachycardia(心动过速心动过速)Thelossof50%severeshockevendealth.第13页/共43页ClinicalmanifestationsFactor13Normaltohigh3Neurologicsystem:Headache,Dizziness,Confusion,decreasedmentalacuity(记忆力衰退),晕厥，注意力不集中，失眠，耳鸣severeanemiaComa4Digestivesystem:Anorexia(食欲不振)，厌食earlymegaloblasticanemiaNausea(恶心)，flatulence(胀气)，diarrhoea(腹泻)orconstipation(便秘)lingualabnormalities(舌的改变)：common1）Endogenous(intra-erythrocytedefects)1.6)影像学检查：钡餐透视，钡灌肠一、DecreasederythropoiesisIDA,SideroblasticanemiaAA,HA,Acuteposthemorragicanemia二、AccelerateddestructionofredcellsHyperplasticanemias(增生性贫血):HemolyticanemiaNormaltohighIncontrast,thegradualloss(evenHb3040g/L)ofthecirculatingredcellmassinapatientwithperniciousanemiamayoccurwithoutanysymptomsatall.第14页/共43页NormaltohighIncontrast,the14Why?Redcell2,3DPG(RBC内内2,3-二磷酸甘油酸）二磷酸甘油酸）Inanemiatheoxyhemoglobindissociationcurveusuallyshifts(右移右移)inamannertoincreasethequantityofoxygenreleasedintissueswithoutoppeciablyalteringthequantityofosygenboundinthelungs.Redcell2,3diphophoglycerate(2,3DPG)regularlyincreasesinanemicpatientstomediatethiseffect.MaximumelevationofRBC2,3DPGincreasesoxygendeliveryonlyabout30percent,butthisisahighlyefficientformofcompensationrequiringnosignificantexpenditureofenergy.第15页/共43页Why?Redcell2,3DPG第15页/共415Theagedpatientsorpatientswithvascularorcardiacdiseasesmaynotstandtomilderanemia.第16页/共43页Theagedpatientsor16Thelevelofanemiaatwhichsymptomsoccurishighlyvariableamongindividualsaswouldbeexpectedfromthewidelydifferingdegreesofphysicalactivity,physicalconditioning,circulatoryadequacy,andsensitivityorstoicismofthepopulation.第17页/共43页Thelevelofanemiaatwhichs17Generalmanifestations:(Nonspecific and reflect tissue hypoxia)Tiredness,fatiguethemostcommon,theearliestsymptomPallorofskinandmucosasharedchracteristicCardiovascularsystems:Palpitationsanddyspneaonexertion,breathlessnesscommonsymptomsValvulaemurmursHb3mAnemiccardicdisease第18页/共43页Generalmanifestations:(Non183Neurologicsystem:Headache,Dizziness,Confusion,decreasedmentalacuity(记忆力衰退记忆力衰退),晕厥，注意力晕厥，注意力不集中，失眠，耳鸣不集中，失眠，耳鸣severeanemiaComa4Digestivesystem:Anorexia(食欲不振食欲不振)，厌食，厌食earlymegaloblasticanemiaNausea(恶心恶心)，flatulence(胀气胀气)，diarrhoea(腹泻腹泻)orconstipation(便秘便秘)lingualabnormalities(舌的改变舌的改变)：common第19页/共43页3Neurologicsystem:Heada195Genitourinarysystem:severeanemiapolyurea(多尿多尿),hypobaricurine(尿比重低尿比重低),proteinuria(蛋白尿蛋白尿):肾小球滤过功能和小肾小球滤过功能和小管分泌及回收功能障碍管分泌及回收功能障碍female:disturbedmenstruation(月经紊月经紊乱乱),性功能减退多见性功能减退多见6Other:7.Manifestationsofunderlyingdisease:第20页/共43页5Genitourinarysystem:severe20Theprocessofcorrectdiagnosisistheoneofdifferentialdiagnosis.Steps:1.ToEstablishthetypeof anemia2.TofindoutthecauseorunderlyingdiseasesofanemiaDiagnosis第21页/共43页Theprocessofcorrectdiagnos211.1.Cause or inducer of anemia:Cause or inducer of anemia:Nutrition,specialhabitsforfoodchangeinstoolhabits:stoolGuaiacsinallprofessioninfluationofsurroundingenvironmentchronicdiseasesmenstruation,marriage一一History第22页/共43页Causeorinducerofanemia:N222.Developing processes,severity 2.Developing processes,severity&complications of anemias&complications of anemiasDurationandonsetofsymptoms3.Important laboratory results,3.Important laboratory results,diagnosis,treatments and their diagnosis,treatments and their effects.effects.第23页/共43页2.Developingprocesses,se23skinandmucosa:pallor,jaundice,petechiaehairandnailsadenopathy(淋巴结肿大淋巴结肿大)hepatomegaly-splenomegalyneurologicabnormalities肛门及妇科盆腔检查肛门及妇科盆腔检查二二Physical examination第24页/共43页skinandmucosa:pallor,jaund24、The hemogram The hemogram（血像）（血像）Routinebloodexam(Hb,RBC)Thereticulocytecount(RC)RC:hyperplasticRC:hypoplastic三、三、Laboratory findings第25页/共43页、Thehemogram（血像）Routineb25Reticulocyte CountIsrequiredintheevaluationofallpatientswithanemiaasitisasimplemeasureofproductionYoungRBCthatstillcontainsasmallamountofRNANormallytake1dayforreticulocytetomature.Underinfluenceofepotakes2-3days1/120thofRBCnormally第26页/共43页ReticulocyteCount第26页/共43页26Absolute Retic countReticcountsarereportedasapercentage:RBCcountxRetic%=Absoultereticcountnormal:(77+23)x109/LAbsoluteReticcountsneedtobecorrectedforearlyrelease(Ifpolychromasiaispresent)Absoluteretic/2(forhctinmid20s)Absoluteretic/3(hct20)第27页/共43页AbsoluteReticcountReticcoun27MCV(themeancorpuscularvolume):themostusefuloftheRBCindices.MCH,MCHC:arerarelyashelpfulastheMCVTheleukocyteandplateletcount:Anemiawithadiminishedleukocyteandplateletcount-pancytopenia-suggestseitherprimarymarrowdisease,megaloblasticanemia,orhypersplenism.Examinationoftheperipheralbloodsmear:第28页/共43页MCV(themeancorpuscularvolum282.Examination of bone marrow2.Examination of bone marrow骨髓涂片检查：主要观察骨髓涂片检查：主要观察BM增生程度，各系统细胞分类计增生程度，各系统细胞分类计数，异常细胞，正常数，异常细胞，正常BM组织有核细胞与脂肪组织各占组织有核细胞与脂肪组织各占1/2，前者增多（尤其是红系）见于增生性贫血，后者增，前者增多（尤其是红系）见于增生性贫血，后者增多代表多代表BM增生低下，见于增生低下，见于AA，骨髓小粒是血液稀释与，骨髓小粒是血液稀释与否的一个重要标志。否的一个重要标志。Examinationofaspiratedsmearsingeneralgivessuperiorcytologicinformationwhilethecorebiopsyprovidescrucialinformationconcerningtheoverallcellularity,aswellasthepresenceoffibrosis,tumor,orgranulomas.Bothproceduresarecomplemantaryandarebestperformedtogetherwhenthediagnosisisindoubt.Bonemarrowironstaining第29页/共43页2.Examinationofbonemarrow293.Some useful ancillary tests3.Some useful ancillary tests1)Stoolsforoccultblood2)Testsforhemolyticanemiasuchas Coombstest3)Testsfornutritionalanemiasuchasserumfolicacid,serumVitaminB12,SI,SF,etc.4)Liverandkidneyfunctiontests5)Immunologictests6)影像学检查：钡餐透视，钡灌肠影像学检查：钡餐透视，钡灌肠第30页/共43页3.Someusefulancillarytests30Treatment一、一、Treatment of the causesThe purpose is the treatment of the underlying disease.GastriccancerPerniciousanemia（恶性贫血）（恶性贫血）（megaloblasticanemia）第31页/共43页Treatment一、Treatmentofthec31二、二、Drugs明确病因之前且忌乱投药明确病因之前且忌乱投药Ironagents;calculationofdosefalicacid,VitB12VitB6Corticosteroids:AIHA，AA,PNHAndrogens:maypromatereythropoiesis:1.)刺激刺激EPO分泌。分泌。2）增强）增强BM对对EPO的效应。的效应。EPO:AnemiacausedbykidneydiseasesImmunosuppressionagentsALG(antilymphocyteglobulin）CsA:AcuteandsevereAA第32页/共43页二、Drugs明确病因之前且忌乱投药第32页/共43页32三、三、TransfusionsAvoidofcomplicationsashepatitisandAIDSWholebloodFrozedredcellsWashedredcellsLeukocytepoorpackedcells（浓缩（浓缩RBC）Chronicanemia:Hb300)lowAnemiaofChronicDxlowlowNormal to highAplasticanemia HighExtremely highNormal to high第36页/共43页LabEvaluationofHypoprolifer36Laboratory findings in anaemiaDrs Shepherd,Dexter,and Rapson Spring 2001第37页/共43页Laboratoryfindingsinanaemia37ScanningElectronmicroscopy:normalredcell第38页/共43页ScanningElectronmicroscopy:第38教学贫血全面版课件