小兒造血和血液特點英文

1、2021/3/102ContentsContentsn Development of hematopoietic systemn Hematopoietic organsn Hematopoietic blood cellsn Characteristic of cell counts and hemoglobinn RBC and Hb leveln Hemoglobinn WBC/Platelet/Blood volumen AnemiaHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/103Development of Development o

2、f Hematopoietic SystemHematopoietic SystemHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/104 HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/105 In the embryo and fetus,constant changes characterize all phases of hematopoiesis. HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/106Development of Development

3、 of Hematopoietic OrgansHematopoietic OrgansFetal hematopoiesisMesoblastic Hematopoiesis Hepatic Hematopoiesis Myeloid Hematopoiesis Hematopoiesis after birth HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/107 Development of Development of Hematopoietic OrgansHematopoietic OrgansFetal Hematopoiesisli

4、ver2021/3/108 SITESTIMEPRODUCMeasoblastic hematopoisisYolk Sac10-14th day3-4wk:primitive blasts10-12wk:ceasedErythroidHepatichematopoiesisLiverSpleen6-8wk:appear12-16wk:active6mo:diminish/ stop at birthErythroidM y e l o i d hematopoiesisBonemarrow4mo:start6mo:increase/steadyafter birth: the onlyEry

5、throidNeutrophilsMacrophagesTable 1. Fetal HematopoiesisTable 1. Fetal HematopoiesisHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/109Development of Development of Hematopoietic OrgansHematopoietic Organs Fetal hematopoiesis Hematopoiesis after birth Bone marrow hematopoiesis Extrmedullary hematopoie

6、sisHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1010Development of Development of Hematopoietic OrgansHematopoietic Organs Hematopoiesis after birth Bone marrow hematopoiesis All blood cells are produced in the marrow after 2nd trimester Newborn and early infancy: red marrow 5-7yr : yellow marrow E

7、xtramedullary hematopoiesisHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1011Development of Development of Hematopoietic OrgansHematopoietic Organs Hematopoiesis after birth Bone marrow hematopoiesis Extramedullary hematopoiesis In diseases status: red cell production hematopoietic tissue. blood pro

8、duction expands to replace fatty marrow. blood cells production extends to extramedullary sites ( liver and spleen). HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1012Development of the Development of the Hematopoietic Blood CellsHematopoietic Blood Cells Pluripotent Stem Cells: which are capable of

9、 both self-renewal and of clonal maturation into all blood cell lineages. Progenitor cells differentiate u n d e r t h e i n f l u e n c e o f hematopoietic growth factorsHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1013Table 2.The Development of Blood CellsPROGENITORCYTOKINESPRODUCCFU-GMG-CSFNUTRO

10、PHILCFU-MegTPOPLTCFU-EBFU-EEPORBCHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1014HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALFig1.Hematopoisis2021/3/1015HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1016Blood Cell Counts and Hemoglobin HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1017Fig2. Peripheral

11、Blood CellsHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1018RBC 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 HOSPITAL2021/3/1019RBC and

12、Hb levelRBC and Hb level 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 HOSPITAL2021/3/1020RBC and Hb levelRBC and Hb level R

13、eticulocytes At Birth: 5% / 10% 1-2mo: fall 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 HOSPITAL2021/3/1021 HEMOGLOBIN. HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL Function transport oxygen. Construc

14、tion iron-containing heme plus globins which is a tetramer made up of two pairs of polypeptide chains, 2021/3/1022 Table3. HEMOGLOBINS HbChains8周周6月月出生出生6-12月月2歲歲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 HOSPITAL2021/3/10

15、23WBC CountsWBC Counts At birth: 20 x 109/L Infant: 12 x 109/L Preschool: 8.0 x 109/LHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1024(%)70淋巴細胞淋巴細胞中性粒細胞中性粒細胞4-6歲歲4-6天天60 50 40 30 20 101 3 5 7 9 日數(shù)日數(shù)1 3 5 7 9 11 歲數(shù)歲數(shù) Fig4. WBC Ratio Fig4. WBC Ratio HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1025P

16、LT & Blood VolumePLT & Blood Volume PLT: 150-250 x 109/L Blood Volume: Term newborn: 85ml/kg Premature infant: 95ml/kg Adult: 75ml/kg Young children: 75-80ml/kgHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1026The Introduction of The Introduction of ANEMIAANEMIAHEMATOLOGY/ONCOLOGY, CHILDRENS

17、 HOSPITAL2021/3/1027Definition of Anemia Definition of Anemia A reduction of the red blood cell volume or hemoglobin concentration below the range of values occurring in healthy persons HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1028AgeAnemia ValuesAnemia DegreeNewborn 145g/L 1-4 mo 90g/LMild: -9

18、0g/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 32-38Microcytic 80 28 32-28H y p o c h r o m i c /Microcytic 80 28 32 Classification-morphologyClassification-morphologyTable 5HEMATOLOGY/ONCOLOGY, CHILDRENS

19、HOSPITAL2021/3/1033Classification- Etiology Reduced capacity to produce red blood cells Hemolysis Blood LossHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1034Classification- etiology Reduced capacity to produce RBC Aplastic anemia Fanconis anemia Acquired aplastic anemia Pure red cell aplasia congen

20、ital hypoplastic anemia (Diamond-Blackfan) Acquired hypoplastic anemiaHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1035Classification- etiology Reduced capacity to produce RBC Marrow Infiltration Leukemia Lymphoma Neuroblastom LCHHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1036Classification-etio

21、logy Reduced capacity to produce RBC Deficiency Syndrome Iron Folate Vitamin B12 Vitamin E Vitamin B6HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1037Classification-etiology Hemolysis Iintrinsic RBC abnormalities Hemoglobinopathies Enzymopathies Membrane disorders extrinsic RBC abnormalities Immuno

22、logic: AIHAHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1038Classification-etiology Hemolysis:intrinsic RBC abnormalities Intrinsic membrane defects Hereditary Spherocytosis: Hemoglobinopathy Thalassemia RBC enzyme defects G6PD defectHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1039Classification-

23、etiology Hemolysis:extrinsic RBC abnormalities Immunologic hemolysis Isoimmune (Rh, ABO in neonate) Autoimmune Hemolytic Anemia (AIHA)HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1040Classification-etiology Blood loss Gastrointestinal bleeding Ankylostomiasis Menstrual TraumaHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1041HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL2021/3/1042HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALHema