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DevelopmentoftheHematopoieticSystem

&

BloodCellCounts

Dr.JieYu,MD.ProfessorThedepartmentofPediatric,Hematology/OncologyHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第1页!ContentsDevelopmentofhematopoieticsystemHematopoieticorgansHematopoieticbloodcellsCharacteristicofcellcountsandhemoglobinRBCandHblevelHemoglobinWBC/Platelet/BloodvolumeAnemiaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第2页!Developmentof

HematopoieticSystem

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第3页! Intheembryoandfetus,constantchangescharacterizeallphasesofhematopoiesis.

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第4页!

Developmentof

HematopoieticOrgansFetalHematopoiesisliver小儿造血和血液特点(英文)共52页,您现在浏览的是第5页!Developmentof

HematopoieticOrgansFetalhematopoiesisHematopoiesisafterbirthBonemarrowhematopoiesisExtrmedullaryhematopoiesisHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第6页!Developmentof

HematopoieticOrgansHematopoiesisafterbirthBonemarrowhematopoiesisExtramedullaryhematopoiesisIndiseasesstatus:

redcellproduction hematopoietictissue.bloodproductionexpandstoreplacefattymarrow.bloodcellsproductionextendstoextramedullarysites(liverandspleen).

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第7页!Table2.TheDevelopmentofBloodCellsPROGENITORCYTOKINESPRODUCCFU-GMG-CSFNUTROPHILCFU-MegTPOPLTCFU-EBFU-EEPORBCHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第8页!HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第9页!Fig2.PeripheralBloodCellsHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第10页!RBCandHblevelPhysiologicalAnemia.

Hemoglobinvaluesinterminfantsdroptotheirlowestmeanof100g/Lat2-3moCausesErythropoietinproductionRedcelllifespan(90/120)Bloodvolume

PreterminfantHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第11页!

HEMOGLOBIN.

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALFunction

transportoxygen.

Constructioniron-containinghemeplusglobinswhichisatetramermadeupoftwopairsofpolypeptidechains,小儿造血和血液特点(英文)共52页,您现在浏览的是第12页!WBCCountsAtbirth:20x109/LInfant:12x109/LPreschool:8.0x109/LHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第13页!PLT&BloodVolumePLT:

150-250x109/LBloodVolume:Termnewborn:85ml/kgPrematureinfant:95ml/kgAdult:75ml/kgYoungchildren:75-80ml/kgHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第14页!DefinitionofAnemia

AreductionoftheredbloodcellvolumeorhemoglobinconcentrationbelowtherangeofvaluesoccurringinhealthypersonsHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第15页!Pathophysiologyincreasedcardiacoutputincreasedoxygenextractionbloodflowtowardvitalorgansandtissues.Inaddition,theconcentrationof2,3-DPGincreaseswithintheRBC.HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

PathophysiologyofAnemia

Selfstudy小儿造血和血液特点(英文)共52页,您现在浏览的是第16页!ManifestationofAnemiaAcuteonsetelevatedpulse,hemicflowmurmur,poorexercisetolerance,headache,excessivesleeping,poorfeeding,andsyncopemayoccur.Slowonsetweakness,tachypnea,shortnessofbreathonexertion,tachycardia,cardiacdilatation,andcongestiveheartfailureHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALSelfstudy小儿造血和血液特点(英文)共52页,您现在浏览的是第17页!Classification-Etiology

ReducedcapacitytoproduceredbloodcellsHemolysisBloodLossTHANKYOUHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第18页!Classification-etiologyReducedcapacitytoproduceRBCMarrowInfiltrationLeukemiaLymphomaNeuroblastomLCHHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第19页!Classification-etiologyHemolysisIintrinsicRBCabnormalitiesHemoglobinopathiesEnzymopathiesMembranedisordersextrinsicRBC

abnormalitiesImmunologic:AIHAHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第20页!Classification-etiology

Hemolysis:extrinsicRBCabnormalitiesImmunologichemolysis

Isoimmune(Rh,ABOinneonate)AutoimmuneHemolyticAnemia(AIHA)HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第21页!MountRaniner,SeattleHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第22页!RBCIndexMCVMeancorpuscularvolumeMCHMeancorpuscularhemoglobinMCHCMeancorpuscularconcertrationHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第23页!HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第24页!HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第25页!HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第26页!小儿造血和血液特点(英文)共52页,您现在浏览的是第27页!

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第28页!Developmentof

HematopoieticOrgans

FetalhematopoiesisMesoblasticHematopoiesisHepaticHematopoiesisMyeloidHematopoiesisHematopoiesisafterbirth

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第29页!

SITESTIMEPRODUCMeasoblastichematopoisisYolkSac10-14thday3-4wk:primitiveblasts10-12wk:ceasedErythroidHepatichematopoiesisLiverSpleen6-8wk:appear12-16wk:active6mo:diminish/stopatbirthErythroidMyeloidhematopoiesisBonemarrow4mo:start6mo:increase/steadyafterbirth:theonlyErythroidNeutrophilsMacrophagesTable1.FetalHematopoiesisHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第30页!DevelopmentofHematopoieticOrgansHematopoiesisafterbirthBonemarrowhematopoiesisAllbloodcellsareproducedinthemarrowafter2ndtrimesterNewbornandearlyinfancy:redmarrow5-7yr:yellowmarrowExtramedullaryhematopoiesisHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第31页!Developmentofthe

HematopoieticBloodCellsPluripotentStemCells:

whicharecapableofbothself-renewalandofclonalmaturationintoallbloodcelllineages.ProgenitorcellsdifferentiateundertheinfluenceofhematopoieticgrowthfactorsHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第32页!HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALFig1.Hematopoisis小儿造血和血液特点(英文)共52页,您现在浏览的是第33页!BloodCellCounts

andHemoglobin

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第34页!RBCandHbLevelAtBirth:

RBC:5-7x1012/LHb:150to230g/L.

Postnatalfall/physiologyanemiaInfancyPreschoolage:RBC:4x1012/LHb:110g/L7-12yr:adultlevelHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第35页!RBCandHblevelReticulocytesAtBirth:5%/10%1-2mo:falldownto0.3%Lateradultlevel:0.5-1.5%NucleatedRedBloodCellsAtbirth:3-10/100WBC;10-20/100WBC1wk:disappearHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第36页!

Table3.HEMOGLOBINS

HbChains8周6月出生6-12月2岁EmbryGower1ζ2ε28周前,3月消失

Gower2α2ε2

Portlanζ2γ2

FetalHbFα2γ2增加90%70%<5%<2%AdultHbAα2β2

5-10%30%>95%

HbA2α2δ2

<1%2-3%<3.5%>95%HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第37页!(%)70淋巴细胞中性粒细胞4-6岁4-6天60

50

40

30

20

1013579

日数1357911

岁数

Fig4.WBCRatio

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第38页!TheIntroductionofANEMIAHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第39页!AgeAnemiaValuesAnemiaDegreeNewborn<145g/L

1-4mo<90g/LMild:-90g/L4-6mo<100g/LModerate:-60g/L6mo-6yr<110g/LSevere:-30g/L6-14yr<120g/LExtremelysevere:<30g/LHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALTable4.ThedefinitionofAnemiaandDegree小儿造血和血液特点(英文)共52页,您现在浏览的是第40页!Fig5:TheoxygendissociationcurveHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALSelfstudy小儿造血和血液特点(英文)共52页,您现在浏览的是第41页!

MCV(fl)MCH(pg)MCHC(%)Normalranges80-9428-3232-38Macrocytic>94>3232-38Normocromic/Normocytic80-9428-3232-38Microcytic<80<2832-28Hypochromic/Microcytic<80<28<32Classification-morphologyTable5HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第42页!

Classification-etiology

ReducedcapacitytoproduceRBCAplasticanemiaFanconi’sanemiaAcquiredaplasticanemiaPureredcellaplasiacongenitalhypoplasticanemia (Diamond-Blackfan)AcquiredhypoplasticanemiaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第43页!Classification-etiology

ReducedcapacitytoproduceRBCDeficiencySyndromeIronFolateVitaminB12VitaminEVitaminB6HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点(英文)共52页,您现在浏览的是第44页!Classification-etiology

Hemolysis:intr

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