小儿造血和血液特点(英文)

1、Development of the Hematopoietic System & Blood Cell CountsDr.Jie Yu, MD. ProfessorThe department of Pediatric, Hematology/Oncology HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALContentsDevelopment of hematopoietic systemHematopoietic organsHematopoietic blood cellsCharacteristic of cell counts and hemoglo

2、binRBC and Hb levelHemoglobinWBC/Platelet/Blood volume AnemiaHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALDevelopment of Hematopoietic SystemHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL In the embryo and fetus,constant changes characterize all phases of hematopoiesis. HE

3、MATOLOGY/ONCOLOGY, CHILDRENS HOSPITALDevelopment of Hematopoietic OrgansFetal hematopoiesisMesoblastic Hematopoiesis Hepatic Hematopoiesis Myeloid Hematopoiesis Hematopoiesis after birth HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL Development of Hematopoietic OrgansFetal HematopoiesisliverSITESTIMEPRODU

4、CMeasoblastic hematopoisisYolk Sac10-14th day3-4wk:primitive blasts10-12wk:ceasedErythroidHepatichematopoiesisLiverSpleen6-8wk:appear12-16wk:active6mo:diminish/ stop at birthErythroidMyeloid hematopoiesisBonemarrow4mo:start6mo:increase/steadyafter birth: the onlyErythroidNeutrophilsMacrophagesTable

5、1. Fetal HematopoiesisHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALDevelopment of Hematopoietic OrgansFetal hematopoiesisHematopoiesis after birthBone marrow hematopoiesisExtrmedullary hematopoiesisHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALDevelopment of Hematopoietic OrgansHematopoiesis after birthBone marr

6、ow hematopoiesisAll 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 hematopoiesisExtramedul

7、lary hematopoiesisIn diseases 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

8、 Stem Cells: which are capable of both self-renewal and of clonal maturation into all blood cell lineages. Progenitor cells differentiate under the influence of hematopoietic growth factorsHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALTable 2.The Development of Blood CellsPROGENITORCYTOKINESPRODUCCFU-GMG-C

9、SFNUTROPHILCFU-MegTPOPLTCFU-EBFU-EEPORBCHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALFig1.HematopoisisHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALBlood Cell Counts and Hemoglobin HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALFig2. Peripheral Blood CellsHEMATOLOGY/ONCOLOGY, CHILD

10、RENS HOSPITALRBC and Hb LevelAt Birth: RBC: 5-7x1012/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

11、lowest mean of 100g/L at 2-3 mo Causes Erythropoietin 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: 3-10/10

12、0 WBC; 10-20/100WBC1wk: disappearHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL 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6月出生6-12月2

13、岁EmbryGower1228周前，3月消失Gower222Portlan22FetalHbF22 增加90%70%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淋巴细胞中性粒细胞4-6岁4-6天6050403020101 3 5 7 9 日数1 3 5 7 9 11 岁数Fig4. WBC Rat

14、io HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALPLT & Blood VolumePLT: 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 An

15、emia A reduction of the red blood cell volume or hemoglobin concentration below the range of values occurring in healthy persons HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALAgeAnemia ValuesAnemia DegreeNewborn 145g/L1-4 mo 90g/LMild: -90g/L4-6 mo 100g/LModerate: -60g/L6 mo-6 yr 110g/LSevere: -30g/L6-14 y

16、r 120g/LExtremelysevere:94 32 32-38Normocromic /Normocytic 80-94 28-32 32-38Microcytic 80 28 32-28Hypochromic /Microcytic 80 28 32 Classification-morphologyTable 5HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification- Etiology Reduced capacity to produce red blood cellsHemolysis Blood LossTHANK YOUH

17、EMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification- etiology Reduced capacity to produce RBCAplastic anemiaFanconis anemiaAcquired aplastic anemiaPure red cell aplasiacongenital hypoplastic anemia (Diamond-Blackfan)Acquired hypoplastic anemiaHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification- e

18、tiologyReduced capacity to produce RBCMarrow InfiltrationLeukemiaLymphomaNeuroblastomLCHHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification-etiology Reduced capacity to produce RBCDeficiency SyndromeIron FolateVitamin B12Vitamin EVitamin B6HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification-etio

19、logyHemolysisIintrinsic RBC abnormalitiesHemoglobinopathiesEnzymopathiesMembrane disordersextrinsic RBC abnormalitiesImmunologic: AIHAHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification-etiologyHemolysis:intrinsic RBC abnormalitiesIntrinsic membrane defectsHereditary Spherocytosis: Hemoglobinopath

20、yThalassemiaRBC enzyme defectsG6PD defectHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification-etiologyHemolysis:extrinsic RBC abnormalitiesImmunologic hemolysis Isoimmune (Rh, ABO in neonate) Autoimmune Hemolytic Anemia (AIHA)HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification-etiologyBlood lossGastrointestinal bleeding AnkylostomiasisMenstrualTraumaHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALMount Raniner, SeattleHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALHEMATOLOGY/ONCOL