教学课件-贫血.ppt

CUF-S(造血干造血干C、红系祖红系祖C)proerythroblast（原始原始RBC）Basophilicnormoblast（早幼早幼RBC）Polychromaticmormoblast（中幼中幼RBC）Orthrochromaticnormoblast（晚幼晚幼RBC）此阶段脱此阶段脱核进入血循环核进入血循环Reticulocyte（网织（网织RBC）经特殊染色见到丝状或网状结构经特殊染色见到丝状或网状结构,保留残余线粒体保留残余线粒体和核糖，仍有继续合成和核糖，仍有继续合成Hb的能力（从早幼的能力（从早幼RBC开始）开始）erythrocyteDefinitionA 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 Factors influencing Hb concentrationSexAgeAltitudeAlterations in plasma volumeClassification Morphologic classificationTypeMCV(um)MCH(PG)MCHC(%)DiagnosisMacrocytic1003232-35Megaloblastic anemiaNormocytic80-10026-3232-35AA,HA,Acute posthemorragic anemiaMicrocytic802632IDA,Sideroblastic anemiaThalassemiasKinetic classification(Classified according to etiology and pathogenesis)一、一、Decreased erythropoiesisDecreased erythropoiesis 1Proliferation and differentiation 2 abnormalities of SCHSC:AA,Fanconisanemia,MDSErythrocyticprogenitors:Pureredcellaplasia,Anemias caused by kidney failureandendocrinedisorders2Marrow infiltration leukemiascarcinomamultiple myeloma myelofibrosisMalignant histocytosis3Production and maturation blockages of differentiating cells DNAsynthesisblockage:VitB12,falicaciddifficiencies,嘌呤和嘧啶嘌呤和嘧啶metabolicdefectsMegaloblasticanemiaHbsynthesisblockage:Hemeproductiondefect:IDAHeptoglobinproductiondefect:Thalassemias4 Unknown reason or several mechanismsSideroblasticanemiaChronicdiseaseanemias(chronicinflummation,infections,尿毒症尿毒症,Hepaticdisorders,neoplasm,connectivetissuedisease,endocrinedisorders)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,HemoglobinopathiesThalassemias2)Extragenous1.Immune HA:AIHA,neonatalHA,mismatchedtransfusion,drug-inducedHA2.2.Mechanical:marchhemoglobinuria,cardiacvalvesprostheses,microangiopathichemolyticdisorders.3.3.Due to chemical,physical or microrganisms:Chemicaltoxin-anddrug-inducedhemolysis,largescaleburnedpatient(severeburnedpatient)Infection-induced,benzene,radiation4.microrganisms:Malaria,virus,etc.5.4.Increased damage of monocyte-macrocyte system:Hypersplenism三三.BloodlossAcuteposthemorrageanemiaChronicbleedingIDAClassifiedaccordindtotheproliferativesituationsofBMHyperplastic anemias(增生性贫血):Hemolytic anemiaAnemia caused by blood lossMegaloblastic anemia,IDA Hypoplastic anemias(增生减低性贫血):AAClinicalmanifestationsFactors influencing symptoms:SeverityofanemiaHb8090g/LsymptomsRapidityofanemiaAbruptlossof20%ofthecirculatingbloodvolumemarkedpallor,posturalhypotensionandtachycardia(心动过速心动过速)Thelossof50%severeshockevendealth.Incontrast,thegradualloss(evenHb3040g/L)ofthecirculatingredcellmassinapatientwithperniciousanemiamayoccurwithoutanysymptomsatall.Why?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.Theagedpatientsorpatientswithvascularorcardiacdiseasesmaynotstandtomilderanemia.Thelevelofanemiaatwhichsymptomsoccurishighlyvariableamongindividualsaswouldbeexpectedfromthewidelydifferingdegreesofphysicalactivity,physicalconditioning,circulatoryadequacy,andsensitivityorstoicismofthepopulation.Generalmanifestations:(Nonspecific and reflect tissue hypoxia)Tiredness,fatiguethemostcommon,theearliestsymptomPallorofskinandmucosasharedchracteristicCardiovascularsystems:Palpitationsanddyspneaonexertion,breathlessnesscommonsymptomsValvulaemurmursHb3mAnemiccardicdisease3Neurologicsystem: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)：common5Genitourinarysystem: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:Theprocessofcorrectdiagnosisistheoneofdifferentialdiagnosis.Steps:1.ToEstablishthetypeof anemia2.TofindoutthecauseorunderlyingdiseasesofanemiaDiagnosis1.Cause or inducer of anemia:Nutrition,specialhabitsforfoodchangeinstoolhabits:stoolGuaiacsinallprofessioninfluationofsurroundingenvironmentchronicdiseasesmenstruation,marriage一一History2.Developing processes,severity&complications of anemiasDurationandonsetofsymptoms3.Important laboratory results,diagnosis,treatments and their effects.skinandmucosa: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、The hemogram（血像）血像）Routinebloodexam(Hb,RBC)Thereticulocytecount(RC)RC:hyperplasticRC:hypoplastic三、三、Laboratory findingsReticulocyte CountNormal:0.002-0.015Is required in the evaluation of all patients with anemia as it is a simple measure of productionYoung RBC that still contains a small amount of RNA Normally take 1 day for reticulocyte to mature.Under influence of epo takes 2-3 days1/120th of RBC normallyAbsolute Retic countRetic counts are reported as a percentage:RBC count x Retic%=Absoulte retic count normal:(77+23)x 109/LAbsolute Retic counts need to be corrected for early release(If polychromasia is present)Absolute retic/2(for hct in mid 20s)Absolute retic/3(hct 20)MCV(themeancorpuscularvolume):themostusefuloftheRBCindices.MCH,MCHC:arerarelyashelpfulastheMCVTheleukocyteandplateletcount:Anemiawithadiminishedleukocyteandplateletcount-pancytopenia-suggestseitherprimarymarrowdisease,megaloblasticanemia,orhypersplenism.Examinationoftheperipheralbloodsmear:2.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.Bonemarrowironstaining3.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Treatment一、一、Treatment of the causesThe purpose is the treatment of the underlying disease.GastriccancerPerniciousanemia（恶性贫血）恶性贫血）（megaloblasticanemia）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三、三、TransfusionsAvoidofcomplicationsashepatitisandAIDSWholebloodFrozedredcellsWashedredcellsLeukocytepoorpackedcells（浓缩浓缩RBC）Chronicanemia:Hb300)lowAnemiaofChronicDxlowlowNormal to highAplasticanemia HighExtremely highNormal to highLaboratory findings in anaemiaDrs Shepherd,Dexter,and Rapson Spring 2001ScanningElectronmicroscopy:normalredcell