小儿造血和血液特点-英文课件

DevelopmentoftheHematopoieticSystem

&

BloodCellCounts

Dr.JieYu,MD.ProfessorThedepartmentofPediatric,Hematology/OncologyHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文ContentsDevelopmentofhematopoieticsystemHematopoieticorgansHematopoieticbloodcellsCharacteristicofcellcountsandhemoglobinRBCandHblevelHemoglobinWBC/Platelet/BloodvolumeAnemiaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Developmentof

HematopoieticSystem

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文 Intheembryoandfetus,constantchangescharacterizeallphasesofhematopoiesis.

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Developmentof

HematopoieticOrgans

FetalhematopoiesisMesoblasticHematopoiesisHepaticHematopoiesisMyeloidHematopoiesisHematopoiesisafterbirth

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文

Developmentof

HematopoieticOrgansFetalHematopoiesisliver小儿造血和血液特点-英文

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Developmentof

HematopoieticOrgansFetalhematopoiesisHematopoiesisafterbirthBonemarrowhematopoiesisExtrmedullaryhematopoiesisHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文DevelopmentofHematopoieticOrgansHematopoiesisafterbirthBonemarrowhematopoiesisAllbloodcellsareproducedinthemarrowafter2ndtrimesterNewbornandearlyinfancy:redmarrow5-7yr:yellowmarrowExtramedullaryhematopoiesisHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Developmentof

HematopoieticOrgansHematopoiesisafterbirthBonemarrowhematopoiesisExtramedullaryhematopoiesisIndiseasesstatus:

redcellproduction

hematopoietictissue

.bloodproductionexpandstoreplacefattymarrow.bloodcellsproductionextendstoextramedullarysites(liverandspleen).

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Developmentofthe

HematopoieticBloodCellsPluripotentStemCells:

whicharecapableofbothself-renewalandofclonalmaturationintoallbloodcelllineages.ProgenitorcellsdifferentiateundertheinfluenceofhematopoieticgrowthfactorsHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Table2.TheDevelopmentofBloodCellsPROGENITORCYTOKINESPRODUCCFU-GMG-CSFNUTROPHILCFU-MegTPOPLTCFU-EBFU-EEPORBCHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALFig1.Hematopoisis小儿造血和血液特点-英文HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文BloodCellCounts

andHemoglobin

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Fig2.PeripheralBloodCellsHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文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RBCandHblevelPhysiologicalAnemia.

Hemoglobinvaluesinterminfantsdroptotheirlowestmeanof100g/Lat2-3moCausesErythropoietinproduction

Redcelllifespan(90/120)Bloodvolume

PreterminfantHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文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

HEMOGLOBIN.

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALFunction

transportoxygen.

Constructioniron-containinghemeplusglobinswhichisatetramermadeupoftwopairsofpolypeptidechains,小儿造血和血液特点-英文

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WBCCountsAtbirth:20x109/LInfant:12x109/LPreschool:8.0x109/LHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文(%)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TheIntroductionofANEMIAHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文DefinitionofAnemia

AreductionoftheredbloodcellvolumeorhemoglobinconcentrationbelowtherangeofvaluesoccurringinhealthypersonsHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文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Pathophysiologyincreasedcardiacoutputincreasedoxygenextractionbloodflowtowardvitalorgansandtissues.Inaddition,theconcentrationof2,3-DPGincreaseswithintheRBC.HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

PathophysiologyofAnemia

Selfstudy小儿造血和血液特点-英文Fig5:TheoxygendissociationcurveHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALSelfstudy小儿造血和血液特点-英文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

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Classification-Etiology

ReducedcapacitytoproduceredbloodcellsHemolysisBloodLossTHANKYOUHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文

Classification-etiology

ReducedcapacitytoproduceRBCAplasticanemiaFanconi’sanemiaAcquiredaplasticanemiaPureredcellaplasiacongenitalhypoplasticanemia (Diamond-Blackfan)AcquiredhypoplasticanemiaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Classification-etiologyReducedcapacitytoproduceRBCMarrowInfiltrationLeukemiaLymphomaNeuroblastomLCHHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Classification-etiology

ReducedcapacitytoproduceRBCDeficiencySyndromeIronFolateVitaminB12VitaminEVitaminB6HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Classification-etiologyHemolysisIintrinsicRBCabnormalitiesHemoglobinopathiesEnzymopathiesMembranedisordersextrinsicRBC

abnormalitiesImmunologic:AIHAHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Classification-etiology

Hemolysis:intrinsicRBCabnormalitiesIntrinsicmembranedefectsHereditarySpherocytosis:HemoglobinopathyThalassemiaRBCenzymedefectsG6PDdefectHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Classification-etiology

Hemolysis:extrinsicRBCabnormalitiesImmunologichemolysis

Isoimmune(Rh,ABOinneonate)AutoimmuneHemolyticAnemia(AIHA)HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文Classification-etiologyBloodlossGastrointestinalbleedingAnkylostomiasisMenstrualTraumaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL小儿造血和血液特点-英文MountRaniner,SeattleHEMATOLOGY/O