小儿造血和血液特点(英文).ppt

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1、Development of the Hematopoietic System&Blood Cell CountsDr.Jie Yu,MD.ProfessorThe department of Pediatric,Hematology/Oncology HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALContentsnDevelopment of hematopoietic systemnHematopoietic organsnHematopoietic blood cellsnCharacteristic of cell counts and hemoglobi

2、nnRBC and Hb levelnHemoglobinnWBC/Platelet/Blood volumen AnemiaHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Hematopoietic SystemHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITAL In the embryo and fetus,constant changes characterize all phases of hematopoiesis.HEMAT

3、OLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Hematopoietic Organs Fetal hematopoiesisMesoblastic Hematopoiesis Hepatic Hematopoiesis Myeloid Hematopoiesis Hematopoiesis after birth HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Hematopoietic OrgansFetalHematopoiesisliverSITESTIMEPRODUCMeaso

4、blastichematopoisisYolkSac10-14thday3-4wk:primitiveblasts10-12wk:ceasedErythroidHepatichematopoiesisLiverSpleen6-8wk:appear12-16wk:active6mo:diminish/stopatbirthErythroidMyeloidhematopoiesisBonemarrow4mo:start6mo:increase/steadyafterbirth:theonlyErythroidNeutrophilsMacrophagesTable 1.Fetal Hematopoi

5、esisHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Hematopoietic OrgansFetal hematopoiesisHematopoiesis after birthBone marrow hematopoiesisExtrmedullary hematopoiesisHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Hematopoietic OrgansHematopoiesis after birthBone marrow hematopoiesisAll

6、blood cells are produced in the marrow after 2nd trimester Newborn and early infancy:red marrow 5-7yr:yellow marrowExtramedullary hematopoiesisHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Hematopoietic OrgansHematopoiesis after birthBone marrow hematopoiesisExtramedullary hematopoiesisIn dis

7、eases status:red cell production hematopoietic tissue.blood production expands to replace fatty marrow.blood cells production extends to extramedullary sites(liver and spleen).HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of the Hematopoietic Blood CellsPluripotent Stem Cells:which are capable o

8、f both self-renewal and of clonal maturation into all blood cell lineages.Progenitor cells differentiate under the influence of hematopoietic growth factorsHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALTable2.TheDevelopmentofBloodCellsPROGENITORCYTOKINESPRODUCCFU-GMG-CSFNUTROPHILCFU-MegTPOPLTCFU-EBFU-EEPORB

9、CHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALFig1.HematopoisisHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALBlood Cell Counts and Hemoglobin HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALFig2.Peripheral Blood CellsHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALRBC and Hb LevelAt Birth:RBC:5-7

10、x1012/L Hb:150 to 230g/L.Postnatal fall/physiology anemiaInfancy Preschool age:RBC:4 x 1012/L Hb:110 g/L 7-12yr:adult levelHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALRBC and Hb levelPhysiological Anemia.Hemoglobin values in term infants drop to their lowest mean of 100g/L at 2-3 mo Causes Erythropoietin

11、production Red cell life span(90/120)Blood volume Preterm infant HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALRBC and Hb levelReticulocytesAt Birth:5%/10%1-2mo:fall down to 0.3%Later adult level:0.5-1.5%Nucleated Red Blood CellsAt birth:3-10/100 WBC;10-20/100WBC1wk:disappearHEMATOLOGY/ONCOLOGY,CHILDRENS HO

12、SPITAL HEMOGLOBIN.HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALFunction transport oxygen.Constructioniron-containing heme plus globins which is a tetramer made up of two pairs of polypeptide chains,Table3.HEMOGLOBINSHbChains8周周6月月出生出生6-12月月2岁岁EmbryGower1228周前,周前,3月消月消失失Gower222Portlan22FetalHbF22增加增加90%70%

13、5%95%HbA2221%2-3%95%HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALWBC CountsAt birth:20 x 109/LInfant:12 x 109/LPreschool:8.0 x 109/LHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITAL(%)70淋巴细胞淋巴细胞中性粒细胞中性粒细胞4-6岁岁4-6天天60504030201013579日数日数1357911岁数岁数Fig4.WBC RatioHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALPLT&Blood VolumePLT:

14、150-250 x 109/LBlood Volume:Term newborn:85ml/kgPremature infant:95ml/kgAdult:75ml/kgYoung children:75-80ml/kgHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALThe Introduction of ANEMIAHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDefinition of Anemia A reduction of the red blood cell volume or hemoglobin concentratio

15、n below the range of values occurring in healthy persons HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALAgeAnemiaValuesAnemiaDegreeNewborn145g/L1-4mo90g/LMild:-90g/L4-6mo100g/LModerate:-60g/L6mo-6yr110g/LSevere:-30g/L6-14yr120g/LExtremelysevere:943232-38Normocromic/Normocytic80-9428-3232-38Microcytic802832-2

16、8Hypochromic/Microcytic802832Classification-morphologyTable5HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-Etiology Reduced capacity to produce red blood cellsHemolysis Blood LossHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiology Reduced capacity to produce RBCAplastic anemiaFanconi

17、s anemiaAcquired aplastic anemiaPure red cell aplasiacongenital hypoplastic anemia (Diamond-Blackfan)Acquired hypoplastic anemiaHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiologyReduced capacity to produce RBCMarrow InfiltrationLeukemiaLymphomaNeuroblastomLCHHEMATOLOGY/ONCOLOGY,CHILDRENS

18、HOSPITALClassification-etiology Reduced capacity to produce RBCDeficiency SyndromeIron FolateVitamin B12Vitamin EVitamin B6HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiologyHemolysisIintrinsic RBC abnormalitiesHemoglobinopathiesEnzymopathiesMembrane disordersextrinsic RBC abnormalitiesImm

19、unologic:AIHAHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiologyHemolysis:intrinsic RBC abnormalitiesIntrinsic membrane defectsHereditary Spherocytosis:HemoglobinopathyThalassemiaRBC enzyme defectsG6PD defectHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiologyHemolysis:extrinsic R

20、BC abnormalitiesImmunologic hemolysis Isoimmune(Rh,ABO in neonate)Autoimmune Hemolytic Anemia(AIHA)HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiologyBlood lossGastrointestinal bleeding AnkylostomiasisMenstrualTraumaHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPIT

21、ALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHematopoiesis Related TermsCFU-GMcolony forming units granulocyte-macrophagesCFU-Megcolony-forming unite-megakaryocyteCFU-Ecolony-forming units-erythroidBFU-Eburst-forming units-erythroidG-CSFcolony-stimulating factorTPOthrombopoietinEPOerythropoietinRBC Index

22、MCVMeancorpuscularvolumeMCHMeancorpuscularhemoglobinMCHCMeancorpuscularconcertrationHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITAL

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