造血系统疾病-小儿造血和贫血(英文)

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1、Development of the Hematopoietic System 10-20/100WBC 1wk: disappearHEMOGLOBIN. Function transport oxygen. Construction iron-containing heme plus globins which is a tetramer made up of two pairs of polypeptide chains, Fig3. Hb structureTable3. HEMOGLOBINSHbChains8周6月出生6-12月2岁EmbryGower1228周前 ,3月消 失Go

2、wer222 Portland22 FetalHbF22 增加90%70%95% HbA222 94 32 32-38Normocromic /Normocytic80-94 28-32 32-38Microcytic 80 28 32-28Hypochromic /Microcytic 80 28 32 Classification-morphologyTable 5Classification- etiology Reduced capacity to produce RBC Aplastic anemia Bone marrow failure Deficiency syndromes

3、Hemolysis Blood LossClassification- etiology Reduced capacity to produce RBC Aplastic anemia Fanconis anemia Acquired aplastic anemia Pure red cell aplasia congenital hypoplastic anemia(Diamond-Blackfan) Acquired hypoplastic anemiaClassification- etiology Reduced capacity to produce RBC Marrow Infil

4、trationLeukemiaLymphomaNeuroblastomLCHClassification-etiology Reduced capacity to produce RBC Deficiency Syndrome Iron Folate Vitamin B12 Vitamin E Vitamin B6Classification-etiology Hemolysis Iintrinsic RBC abnormalities Hemoglobinopathies Enzymopathies Membrane disorders extrinsic RBC abnormalities

5、 Immunologic: AIHAClassification-etiology Hemolysis:intrinsic RBC abnormalities Intrinsic membrane defects Hereditary Spherocytosis: Hemoglobinopathy Thalassemia RBC enzyme defects G6PD defectClassification-etiology Hemolysis:extrinsic RBC abnormalities Immunologic hemolysis Isoimmune (Rh, ABO in ne

6、onate) Autoimmune Hemolytic Anemia (AIHA)Classification-etiology Blood loss Gastrointestinal bleeding Ankylostomiasis Menstrual TraumaHematopoiesis Regulation Related termsCFU-GM colony forming units granulocyte-macrophages CFU-Meg colony-forming unite -megakaryocyte CFU-E colony-forming units -erythroid BFU-E burst-forming units -erythroid G-CSF colony-stimulating factor TPO thrombopoietin EPO erythropoietinRBC Index MCV Mean corpuscular volume MCH Mean corpuscular hemoglobin MCHC Mean corpuscular concertration

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