教學(xué)課件-貧血

CUF-S(造血干C、紅系祖C)

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proerythroblast（原始RBC）

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Basophilicnormoblast（早幼RBC）

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Polychromaticmormoblast（中幼RBC）

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Orthrochromaticnormoblast（晚幼RBC）此階段脫核進入血循環(huán)

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Reticulocyte（網(wǎng)織RBC）

經(jīng)特殊染色見到絲狀或網(wǎng)狀結(jié)構(gòu),保留殘余線粒體和核糖，仍有接著合成Hb的實力（從早幼RBC起先）

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erythrocyteDefinitionAreductionbelownormalinthe

concentrationofhemoglobin,themassofredbloodcellsand/orthehematocritintheblood.InmenHb<120g/L RBC<4.5×1012/L HCT<0.42InwomenHb<110g/LRBC<4.0×1012/L HCT<0.37FactorsinfluencingHbconcentrationSexAgeAltitudeAlterationsinplasmavolume★Classification

MorphologicclassificationKineticclassification

(Classifiedaccordingtoetiologyandpathogenesis)一、Decreasederythropoiesis

ProliferationanddifferentiationabnormalitiesofSC HSC:AA,Fanconisanemia,MDS Erythrocyticprogenitors:Pureredcellaplasia, Anemiascausedbykidneyfailureand endocrinedisordersMarrowinfiltration

leukemias

carcinoma

multiplemyeloma

myelofibrosis

MalignanthistocytosisProductionandmaturationblockagesofdifferentiatingcells

DNAsynthesisblockage:VitB12,falic aciddifficiencies,嘌呤和嘧啶 metabolicdefects→Megaloblastic anemia

Hbsynthesisblockage:

Hemeproductiondefect:IDA

Heptoglobinproductiondefect: Thalassemias4UnknownreasonorseveralmechanismsSideroblasticanemia

Chronicdiseaseanemias(chronicinflummation,infections,尿毒癥,Hepaticdisorders,neoplasm,connectivetissuedisease,endocrinedisorders)二、Accelerateddestructionofredcells

1）Endogenous(intra-erythrocytedefects)1.Membranedefectsoferythrocytes:

Hereditary:Hereditaryspherocytosis(HS)

Hereditasyelliplocytosis(HE) Acquired:PNH

2.Enzymedefects

Glucose--6--PhosphateDehydrogenase (G6PD)deficiency

Pyruvatekinasedeficiency3.Abnormalhaptogllbinsynthesis:

Sicklecellanemia,Hemoglobinopathies

Thalassemias2)ExtragenousImmuneHA:AIHA,neonatalHA,mismatchedtransfusion,

drug-inducedHA2.Mechanical:marchhemoglobinuria,cardiacvalvesprostheses,microangiopathichemolyticdisorders.3.

Duetochemical,physicalormicrorganisms:

Chemicaltoxin-anddrug-inducedhemolysis,

largescaleburnedpatient(severeburnedpatient)

Infection-induced,benzene,radiation microrganisms:Malaria,virus,etc.4.Increaseddamageofmonocyte-macrocytesystem:Hypersplenism三.

Bloodloss◆Acuteposthemorrageanemia◆Chronicbleeding→IDAClassifiedaccordindtotheproliferativesituationsofBMHyperplasticanemias(增生性貧血):

Hemolyticanemia Anemiacausedbybloodloss Megaloblasticanemia,IDA

Hypoplasticanemias(增生減低性貧血):AAClinicalmanifestationsFactorsinfluencingsymptoms:

Severityofanemia Hb<80~90g/L→symptomsRapidityofanemia

Abruptlossof20%ofthecirculating bloodvolume→markedpallor, posturalhypotensionandtachycardia (心動過速)

Thelossof50%→severeshockeven dealth.

Incontrast,thegradualloss(evenHb30~40g/L)ofthecirculatingredcellmassinapatientwithperniciousanemiamayoccurwithoutanysymptomsatall.Why?

Redcell2,3DPG

(RBC內(nèi)2,3-二磷酸甘油酸）↑ Inanemiatheoxyhemoglobindissociationcurveusuallyshifts(右移)inamannertoincreasethequantityofoxygenreleasedintissueswithoutoppeciablyalteringthequantityofosygenboundinthelungs.Redcell2,3diphophoglycerate(2,3DPG)regularlyincreasesinanemicpatientstomediatethiseffect.MaximumelevationofRBC2,3DPGincreasesoxygendeliveryonlyabout30percent,butthisisahighlyefficientformofcompensationrequiringnosignificantexpenditureofenergy.

Theagedpatientsorpatientswithvascularorcardiacdiseasesmaynotstandtomilderanemia.Thelevelofanemiaatwhichsymptomsoccurishighlyvariableamongindividualsaswouldbeexpectedfromthewidelydifferingdegreesofphysicalactivity,physicalconditioning,circulatoryadequacy,andsensitivityorstoicismofthepopulation.１．Generalmanifestations:

(Nonspecificandreflecttissuehypoxia)

Tiredness,fatigue→themostcommon,the earliestsymptom

Pallorofskinandmucosa→shared

chracteristic

２．Cardiovascularsystems:

Palpitationsanddyspneaonexertion,

breathlessness→commonsymptoms

Valvulaemurmurs

Hb<30g/L,>3m→Anemiccardicdisease3．Neurologicsystem:

Headache,Dizziness,Confusion,decreasedmentalacuity(記憶力衰退),暈厥，留意力不集中，失眠，耳鳴．

severeanemia→Coma

4．Digestivesystem:

Anorexia(食欲不振)，厭食→earlymegaloblasticanemia

Nausea(惡心)，flatulence(脹氣)，diarrhoea(腹瀉)orconstipation(便秘)

lingualabnormalities(舌的變更)：common5．Genitourinarysystem:

severeanemia→polyurea(多尿),hypobaricurine(尿比重低),proteinuria(蛋白尿):腎小球濾過功能和小管分泌及回收功能障礙

female:disturbedmenstruation(月經(jīng)紊亂),性功能減退多見

6．Other:

7.Manifestationsofunderlyingdisease:Theprocessofcorrectdiagnosisistheoneofdifferentialdiagnosis.

Steps:

1.ToEstablishthetypeof anemia

2.Tofindoutthecauseor underlyingdiseasesofanemiaDiagnosisCauseorinducerofanemia:

Nutrition,specialhabitsforfoodchangeinstoolhabits:stoolGuaiacsinall

profession

influationofsurroundingenvironmentchronicdiseases

menstruation,marriage一．History

2.Developingprocesses,severity&complicationsofanemias

Durationandonsetofsymptoms

3.Importantlaboratoryresults,diagnosis,treatmentsandtheireffects.

skinandmucosa:pallor,jaundice, petechiae

hairandnails

adenopathy(淋巴結(jié)腫大)hepatomegaly-splenomegaly

neurologicabnormalities

肛門及婦科盆腔檢查二．Physicalexamination１、Thehemogram（血像） Routinebloodexam(Hb,RBC)

Thereticulocytecount(RC)

RC↑:hyperplastic

RC↓:hypoplastic三、LaboratoryfindingsReticulocyteCountNormal:0.002-0.015IsrequiredintheevaluationofallpatientswithanemiaasitisasimplemeasureofproductionYoungRBCthatstillcontainsasmallamountofRNANormallytake1dayforreticulocytetomature.Underinfluenceofepotakes2-3days1/120thofRBCnormallyAbsoluteReticcountReticcountsarereportedasapercentage:RBCcountxRetic%=Absoultereticcountnormal:(77+23)x109/LAbsoluteReticcountsneedtobecorrectedforearlyrelease(Ifpolychromasiaispresent)Absoluteretic/2(forhctinmid20’s)Absoluteretic/3(hct<20)MCV(themeancorpuscularvolume):the mostusefuloftheRBCindices.

MCH,MCHC:arerarelyashelpfulasthe MCV

Theleukocyteandplateletcount:

Anemiawithadiminishedleukocyteandplateletcount-pancytopenia-suggestseitherprimarymarrowdisease,megaloblasticanemia,orhypersplenism.

Examinationoftheperipheralbloodsmear:2.Examinationofbonemarrow骨髓涂片檢查：主要視察BM增生程度，各系統(tǒng)細胞分類計數(shù)，異樣細胞，正常BM組織有核細胞與脂肪組織各占1/2，前者增多（尤其是紅系）見于增生性貧血，后者增多代表BM增生低下，見于AA，骨髓小粒是血液稀釋與否的一個重要標記。

Examinationofaspiratedsmearsingeneralgivessuperiorcytologicinformationwhilethecorebiopsyprovidescrucialinformationconcerningtheoverallcellularity,aswellasthepresenceoffibrosis,tumor,orgranulomas.Bothproceduresarecomplemantaryandarebestperformedtogetherwhenthediagnosisisindoubt.Bonemarrowironstaining3.Someusefulancillarytests1)

Stoolsforoccultblood2)

Testsforhemolyticanemiasuchas Coombs’test3)

Testsfornutritionalanemiasuchasserum folicacid,serumVitaminB12,SI,SF,etc.4)

Liverandkidneyfunctiontests5)

Immunologictests6)

影像學(xué)檢查：鋇餐透視，鋇灌腸Treatment一、TreatmentofthecausesThepurposeisthetreatmentoftheunderlyingdisease.

Gastriccancer→Pernici