贫血概述课件

OVERVIEWOFANEMIAWHATISBLOOD?WHATDOESBLOODDO?VIACIRCULATORYSYSTEM

Bringoxygen&nutrientstocells

TakeawayCO2&otherwastesawayfromcells

CarryWBCtotheplaceswheretheyareneeded

Bringhormones&antibodiestotargetedorgans

ortissuesorcellsRedBloodCells(Erythrocytes)MostabundantbloodcellsContainstheredpigmenthemoglobinwhichbindsandtransportsO2andCO2EachRBCisabiconcavediscErythrocytesWhyabiconcavedisc?ProvidesalargesurfaceareaforO2entry/exitEnablesthemtobendandflexwhenenteringsmallcapillariesRBCslackanucleusandmostorganelles.InsteadtheyaresimplymembranousbagsofhemoglobinHere,wehaveanRBCbendingtofitthruasmallcapillaryAN=without

EMIA=blood

ANEMIA=withoutblood1DEFINITIONOFANEMIAANEMIA:Reductionbelownormallimitsofthetotalredcellmass

Reductioninhemoglobinconcentrationofblood（Hb）

Reductionin

numbersofredbloodcells（RBC）

Reductionbelownormalinvolumeofpackedcells(hematocrit，Hct)

外周血液单位体积中血红蛋白浓度，红细胞计数和(或)红细胞比容低于正常最低值者称为贫血。其中血红蛋白浓度最常用和可靠。DEFINITIONOFANEMIAGenderHb(g/L)RBC(×1012/L)HctMale＜120＜4.5＜0.42Female*＜110＜4.0＜0.37

FemaleinpregnancyisdefinedasHb＜100g/L.Hct

＜0.30NormalValuesInfluentialFactorsofAnemiaAge

SexAltitudeAlterationsinplasmavolume

Bloodconcentration:diarrhrea,burningetc.

Blooddilution:congestiveheartfailure，etc.Bloodloss2EtiologyandPathogenesisAlwaysbearinmind:

Anemiaitselfisnotadisease

Anemiaisasignofmanydiseases

Diagnosisofanemiaismerelythefirststepto

theprocessofsearchingforitscausesEtiologyandPathogenesisanemiaDecreasedproductionIncreaseddestruction

Bloodloss

NormalHematopoiesisFeaturesofHematopoieticStemCell

Self-renewal:maintenanceofstabilizedpoolsize

Multipotentialdifferentiation:myeloid&lymphoidlineages

Existancein

BONEMARROW

and

PERIPHERALBLOODErythropoiesisErythropoietin,EpoEtiologyandPathogenesisREDUCEDPRODUCTIONOFRBC

Marrowfailure:reductioninstemcell

Inherited:BMFS(Fanconianemiaetc.)

Acquired:Aplasticanemia

Ineffectiveerythropoiesis:MDS

Marrowsuppression:cytotoxicchemotherapiesEtiologyandPathogenesisREDUCEDPRODUCTIONOFRBC

Marrowinfiltration:tumors,fibrosisetc.

Decreasedgrowthfactors:renalfailure(Epo)

Abnormalityofhematopoieticmicroenvironment

Shortageinhemopoieticmaterials:

folicacid,Vit.B12deficiency(megaloblastic)

irondeficiencyanemia(microcytic)EtiologyandPathogenesisINCREASEDDESTRUCTIONOFRBC

Hemolyticanemia(shortenedlife-spanofRBC)

Intrinsicdeficiencies:

Inherited

Membrane:spherocytosis,elliptocytosis,etc.

Enzymes:G-6PDdeficiency

Hemoglubin:thalassemia,hemoglobinopathy

Acquired

Paroxysmalnocturnalhemoglobinuria（PNH）EtiologyandPathogenesis

Extrinsicfactors:

Immunerelated:antibody-mediated

nonImmunerelated:

Physical:burning,etc.

Chemical:toxics,medicine,etc.

Biological:bacterials,viruses,vipervenometc.

EtiologyandPathogenesisBLOODLOSS

Acutebloodloss

Chronicbloodloss3ClassificationofAnemiacytometricclassificationTYPESMCV(fl)MCH(pg)MCHC(%)Macrocytic>100>3231-35Normocytic80-10026-3231-35Puremicrocytic<80<2631-35Microcyt-hypochr<80<26<26MCV=MeanCellVolumeMCH=MeanCellHemoglobinMCHC=MeanCellHemoglobinConcentrationMorphologicalclassification---人工检测Cytometricclassification（细胞计量学分类）---自动血细胞分析仪检测ClassificationofAnemiaGradingofAnemiaTYPESHemoglobin(g/L)Grade1(mild)>90-normallowerlimitGrade2(moderate)60-90Grade3(severe)30-60Grade4(verysevere)<30*

life-threateninganemiaPhysiopathologicalClassificationDecreasedRBCProductionIncreasedRBCDestruction

Marrowfailure

Ineffectivehematopoiesis

Marrowsuppression:

Marrowinfiltration:

AbnormalDNAsynthesis

AbnormalHbsynthesis

Deregulationoferytheropoiesis

Unknownormultiplemechanisms

IntrinsicRBCabnormalities

ExtrinsicabnormalityBloodLoss

Acutebloodloss

ChronicbloodlossPhysiopathologicalClassificationMarrowfailure

Aplasticanemia(AA)

Fanconianemia（FA）

Pureredcellaplasia(PRCA)

Anemiaduetorenalfailure

Anemiaduetoendocrinedisorders

CongenitaldyserythropoieticanemiaDecreasedRBCProductionPhysiopathologicalClassificationIneffectivehematopoiesis

Myelodysplasticsyndrome(MDS)

Congenitaldyserythropoieticanemia

MegaloblasticanemiaDecreasedRBCProductionPhysiopathologicalClassificationMarrowinfiltrationLeukemiacarcinomametastasistobonemarrowmultiplemyeloma

myelofibrosis

DecreasedRBCProductionPhysiopathologicalClassificationAbnormalDNASynthesis

VitaminB12deficiency

Folicaciddeficiency

Congenitaloracquireddefectsinpurineand

pyrimidinemetabolismDecreasedRBCProductionPhysiopathologicalClassificationAbnormalHbSynthesis

Irondeficiencyanemia

Congenitalatransferrinemia

Thalassemiaandhemoglobinopathy

DecreasedRBCProductionPhysiopathologicalClassificationDeregulationofErytheropoiesis

Lowoxygenaffinityhemoglobinopathies

Highoxygenaffinityhemoglobinopathies

DecreasedRBCProductionPhysiopathologicalClassificationDecreasedRBCProductionUnknownorMultipleMechanisms

Anemiaofchronicdisorders(ACD)

Anemiaduetonutritionaldeficiencies

SideroblasticanemiaPhysiopathologicalClassificationIncreasedRBCDestructionIntrinsicabnormalitiesofRBC

MembranedefectsofRBC

Hereditaryspherocytosis

Hereditaryelliptocytosis

Paroxysmalnocturnalhemaglobinuria

EnzymedeficiencyofRBC

Glucose-6-phosphatedehydrogenase(G-6-PD)deficiency

Pyruvate

kinase(PK)deficiency

PhysiopathologicalClassificationIncreasedRBCDestructionIntrinsicabnormalitiesofRBC

Globinabnormality(Hempglobinopathy)

Abnormalhemaglobinopathies

Disturbanceofglobinsynthesis(Thalassemia)PhysiopathologicalClassificationIncreasedRBCDestructionExtrinsicabnormalities

Antibody-mediated(Auto-orAllo-immune)

Autoimmunehemolyticanemia(AIHA)withwarm-reactingantibody

Cryopathichemolyticdisorders

Drug-relatedantibody-mediatedHA

AlloimmunehemolyticanemiaofnewbornPhysiopathologicalClassificationIncreasedRBCDestructionExtrinsicabnormalities

PhysicalorMechanical

Burning

Marchhemaglobinuria

Traumaticcardiachemolyticanemia

Microangiopathichemolyticanemia

Chemical

Toxicchemicals

Medicationordrugs

Biological

Pathogens(bacterials,viruses,parasites,etc.)

Vipervenom

Hyperactivityofmonocyte-macrophagesystem

Hypersplenism

PhysiopathologicalClassificationBloodLoss

Acute

Chronic4ManifestationsofAnemiaClinicalpictureofanemiaconsistsoftwocomponents:

underlyingdisease

anemiaitself

Manifestationsofanemiadependonthefactorsasfollows:

Loweredoxygen-carryingcapacity

Changeinbloodvolume

Velocityofdevelopingthetwoabove-mentionedfactors

Patient’sabilitytocompensateforanemiaSeverityofanemia

Hb<70g/L→symptomsbeginRapidityofanemia

Abruptlossof20%ofthecirculatingbloodvolume→markedpallor,hypotensionandtachycardia

Thelossof50%→severeshockevendeath.

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

CompensatorymechanismRedcell2,3DPG

(diphosphoglycericacid）↑

theoxyhemoglobindissociationcurverightshiftincreasethequantityofoxygenreleasedintissues

MaximumelevationofRBC2,3DPGincreasesoxygendeliveryonlyabout30%,butthisisahighlyefficientformofcompensationrequiringnosignificantexpenditureofenergy.

DPGCompensatorymechanismSelectivecontractionofbloodvessels,sothatmorebloodflowstocriticalorganSkinandrenalbloodflowreductionIncreaseincardiacoutputSystemicManifestationsofAnemiaSkinandMucosaPALLOR-mostfrequentsign

-Race

-Pigmentation

-

Densityanddilationofsuperficialbloodvessels

-

EdemaObservationoforalmucosa,conjunctiva,lipandnail-bedismorereliableSystemicManifestationsofAnemiaRespiratory&CirculationSystemsSymptomsSigns

Tachycardia

Dyspneaonexertion

Systolicheartmurmur

Non-specificECGchanges(T&ST)

Hb<30g/L,>3m→AnemiccardicdiseaseSystemicManifestationsofAnemiaNeurologic&MuscularSystems

Headache

Dizziness

Tinnitus

Scotoma

Faintness

Lassitude

Poormemory

Weakness

Fatigue

Unabletoconcentrate

SystemicManifestationsofAnemiaAlimentarySystemSymptomsSigns

Lossofappetite

nausea

Fullness

Abdominaldiscomfort

Constipationordiarrhea

Stomatitis

Atrophyofpapilla

Dysphagia(rare)SystemicManifestationsofAnemiaUrogenitalSystemSymptomsLabfindings

Polyuria

Menorrhea

MenostasisIIrregularmensis

Lossoflibido

Proteinuria(mild)SystemicManifestationsofAnemiaMiscellaneous

Low-gradefever(hypermetabolism)

Jaundice(hemolytic)

Hemoglobinuria(intravascularhemolysis)

Hemoglobinemia5DiagnosisofAnemiaHistory

Onset

Course

Dietarypattern

Medication

Exposure

Bleedingtendency

Chronicdisease

Changeinbodyweight

Mensis

Reproduction

Tarrystool

Darkurine

Fever

FamilyDiagnosisofAnemiaPhysicalExamination

Skinandmucosa(color,rash,ulceration)

Spoonshapednail

Lymphnodes,spleen,liver

SternaltendernessDiagnosisofAnemiaLaboratoryTestsScreeningtests

Completecellcount(CBC)

reticulocyte

Morphologyofperipheralbloodsmear

Bonemarrowexamination(ifneeded)Diagnostictests(wheneverindicated)Thereticcount(RC)

RC↑:hyperplastic

RC↓:hypoplasticBloodSmearInterpretation:NormalMicrocyteOvalTargetSpherocyteHeinzbodySchistocyteNucleatedRBCPolychromaticTeardropNormal,RBC's.Theyhaveazoneofcentralpallor(about1/3thesizeoftheRBC).Inthecenterofthefieldareabandneutrophilontheleftandasegmentedneutrophilontheright.

TheRBC'sherearesmallerthannormalandhaveanincreasedzoneofcentralpallor.Thisisindicativeofahypochromic(lesshemoglobinineachRBC)microcytic(smallersizeofeachRBC)anemia.Thereisalsoincreasedanisocytosis(variationinsize)andpoikilocytosis(variationinshape).DiagnosisofAnemiaSummary

Historytakingisessentialforapproachtodiagnosis

FocusedPEtofindpositivesigns

Judiciouschoosingoflabtestsensurepathologicclassification

LowMegaloblasticanemiaLowHb=AnemiaMCVLowmicrocyticNormalnormocyticHighmacrocyticMeasureFerritinLowNormal/highIrondefAnemiaAnemiaofchronicdisease/CongenitalHbdis.Measure

ReticshighlowMarrowfailureRenalfailureAnemiaofchronicdiseaseHemolyticanemiaorbloodlossMeasureB12+folateNormal6TreatmentofAnemiaTreatmentofthecauses

Thepurposeisthetreatmentoftheunderlyingdisease.

Gastriccancer→anemia

TreatmentofAnemiaSupportiveCareTransfusion(Chronicanemia:<60g/L；Abruptlossof30%ofthecirculatingbloodvolume)

–wholeblood(fewindications)–bloodcomponent(inmostcases)

AntibiotictherapyifindicatedTreatmentofAnemiaSupplementofNutrientsorFac