小儿造血和血液特点-英文-课件.ppt

1、Development of the Development of the Hematopoietic SystemHematopoietic System&Blood Cell CountsBlood Cell Counts Dr.Jie Yu,MD.Professor The department of Pediatric,Hematology/Oncology HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALContentsContentsn Development of hematopoietic systemn Hematopoietic organsn

2、Hematopoietic blood cellsn Characteristic of cell counts and hemoglobinn RBC and Hb leveln Hemoglobinn WBC/Platelet/Blood volumen AnemiaHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Development of Hematopoietic SystemHematopoietic SystemHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITAL HEMATOLOGY/ONCOLO

3、GY,CHILDRENS HOSPITAL In the embryo and fetus,constant changes characterize all phases of hematopoiesis.HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Development of Hematopoietic OrgansHematopoietic Organs Fetal hematopoiesis Mesoblastic Hematopoiesis Hepatic Hematopoiesis Myeloid Hematopoies

4、is Hematopoiesis after birth HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITAL Development of Development of Hematopoietic OrgansHematopoietic OrgansFetal Hematopoiesisliver SITESTIMEPRODUCMeasoblastic hematopoisisYolk Sac10-14th day3-4wk:primitive blasts10-12wk:ceasedErythroidHepatichematopoiesisLiverSpleen6-

5、8wk:appear12-16wk:active6mo:diminish/stop at birthErythroidM y e l o i d hematopoiesisBonemarrow4mo:start6mo:increase/steadyafter birth:the onlyErythroidNeutrophilsMacrophagesTable 1.Table 1.Fetal HematopoiesisFetal HematopoiesisHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Development of Hem

6、atopoietic OrgansHematopoietic Organs Fetal hematopoiesis Hematopoiesis after birth Bone marrow hematopoiesis Extrmedullary hematopoiesisHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Development of Hematopoietic OrgansHematopoietic Organs Hematopoiesis after birth Bone marrow hematopoiesis Al

7、l blood cells are produced in the marrow after 2nd trimester Newborn and early infancy:red marrow 5-7yr:yellow marrow Extramedullary hematopoiesisHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDevelopment of Development of Hematopoietic OrgansHematopoietic Organs Hematopoiesis after birth Bone marrow hematop

8、oiesis Extramedullary hematopoiesis In 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 Development of the Hemato

9、poietic Blood CellsHematopoietic Blood Cells Pluripotent 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 Develop

10、ment of Blood CellsPROGENITORCYTOKINESPRODUCCFU-GMG-CSFNUTROPHILCFU-MegTPOPLTCFU-EBFU-EEPORBCHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALFig1.HematopoisisHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALBlood Cell Counts and Hemoglobin HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALFig2.

11、Peripheral Blood CellsHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALRBC and Hb LevelRBC and Hb Level At Birth:RBC:5-7x1012/L Hb:150 to 230g/L.Postnatal fall/physiology anemia Infancy Preschool age:RBC:4 x 1012/L Hb:110 g/L 7-12yr:adult levelHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALRBC and Hb levelRBC and Hb le

12、vel Physiological Anemia.Hemoglobin values in term infants drop to their 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 levelRBC and Hb level Reticulocytes At Birth:5%/10%1-2mo:fa

13、ll down to 0.3%Later adult level:0.5-1.5%Nucleated Red Blood Cells At birth:3-10/100 WBC;10-20/100WBC 1wk:disappearHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITAL HEMOGLOBINHEMOGLOBIN.HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITAL Function transport oxygen.Construction iron-containing heme plus globins which is a te

14、tramer made up of two pairs of polypeptide chains,Table3.HEMOGLOBINSTable3.HEMOGLOBINS HbChains8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 Gower222 Portlan22 FetalHbF22 增加增加90%70%5%95%HbA222 1%2-3%95%HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALWBC CountsWBC Counts At birth:20 x 109/L Infant:12 x 109/L

15、Preschool: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60 50 40 30 20 101 3 5 7 9 日数日数1 3 5 7 9 11 岁数岁数 Fig4.WBC RatioFig4.WBC Ratio HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALPLT&Blood VolumePLT&Blood Volume PLT:150-250 x 109/L Blood Volume:Term newborn:85ml/kg Prema

16、ture infant:95ml/kg Adult:75ml/kg Young children:75-80ml/kgHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALThe Introduction of The Introduction of ANEMIAANEMIAHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALDefinition of AnemiaDefinition of Anemia A reduction of the red blood cell volume or hemoglobin concentration bel

17、ow the range of values occurring in healthy persons HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALAgeAnemia ValuesAnemia DegreeNewborn 145g/L 1-4 mo 90g/LMild:-90g/L4-6 mo 100g/LModerate:-60g/L6 mo-6 yr 110g/LSevere:-30g/L6-14 yr 120g/LExtremelysevere:94 32 32-38Normoc romi c/Normocytic 80-94 28-32 32-38Mic

18、rocytic 80 28 32-28H y p o c h r o m i c/Microcytic 80 28 32 Classification-morphologyClassification-morphologyTable 5HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-Etiology Reduced capacity to produce red blood cells Hemolysis Blood LossHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-eti

19、ology Reduced capacity to produce RBC Aplastic anemia Fanconis anemia Acquired aplastic anemia Pure red cell aplasia congenital hypoplastic anemia (Diamond-Blackfan)Acquired hypoplastic anemiaHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiology Reduced capacity to produce RBC Marrow Infiltr

20、ation Leukemia Lymphoma Neuroblastom LCHHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiology Reduced capacity to produce RBC Deficiency Syndrome Iron Folate Vitamin B12 Vitamin E Vitamin B6HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiology Hemolysis Iintrinsic RBC abnormalities H

21、emoglobinopathies Enzymopathies Membrane disorders extrinsic RBC abnormalities Immunologic:AIHAHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiology Hemolysis:intrinsic RBC abnormalities Intrinsic membrane defects Hereditary Spherocytosis:Hemoglobinopathy Thalassemia RBC enzyme defects G6PD

22、defectHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiology Hemolysis:extrinsic RBC abnormalities Immunologic hemolysis Isoimmune(Rh,ABO in neonate)Autoimmune Hemolytic Anemia(AIHA)HEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALClassification-etiology Blood loss Gastrointestinal bleeding Ankylostomia

23、sis Menstrual TraumaHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHematopoiesis Related TermsCFU-GMcolony forming units granulocyte-macrophagesCFU-Megcolony-forming unite-megakaryocyteCFU-Ecolony-forming units-erythroidBFU-Eburst-fo

24、rming units-erythroid G-CSF colony-stimulating factor TPO thrombopoietin EPO erythropoietinRBC Index MCV Mean corpuscular volume MCH Mean corpuscular hemoglobin MCHC Mean corpuscular concertrationHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY,CHILDRENS HOSPITAL