复旦大学：《儿科学》课程教学资源（PPT课件讲稿，双语版）第六讲 血液系统疾病

Diseases of the blood in Children 血液系统疾病 Associated Professor Gao Yijing Children s Hospital, Fudan University 复旦大学附属儿科医院

Associated Professor Gao Yijing Children`s Hospital, Fudan University Diseases of the Blood in Children 血液系统疾病

Diseases of the blood in children Anemias Nutritional anemias(Iron deficiency iron) Hereditary spherocytosis Glucose-6-phosphate dehydrogenase(G-6-PD) deficiency Thalassemia Bleeding disorders(Hemorrhagic diseases) ITP Hemophilia Neoplastic diseases 回复旦大学上海医学院儿科学系

Diseases of the Blood in Children • Anemias Nutritional anemias (Iron deficiency iron) Hereditary spherocytosis Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency Thalassemia • Bleeding disorders (Hemorrhagic diseases) ITP Hemophilia • Neoplastic diseases

Development of the Hematopoietic System Blood formation first can be recognized as early as 3rd wk after conception By 2 mo active hematopoiesis is established in the liver which is the main site of blood formation during the middle portion of fetal life After about 6 mo hematopoiesis shifts gradually to the medullary spaces, and by birth most blood formation normally takes place in bone marrow Extramedullary hematopoiesis 回复旦大学上海医学院儿科学系

Development of the Hematopoietic System • Blood formation first can be recognized as early as 3rd wk after conception • By 2 mo active hematopoiesis is established in the liver, which is the main site of blood formation during the middle portion of fetal life • After about 6 mo hematopoiesis shifts gradually to the medullary spaces, and by birth most blood formation normally takes place in bone marrow Extramedullary hematopoiesis

Hematologic Values During Infancy and Childhood Normal ranges for peripheral blood counts vary significantly with age Red blood cells Physiologic anemia Hemoglobin White blood cells 回复旦大学上海医学院儿科学系

Hematologic Values During Infancy and Childhood • Normal ranges for peripheral blood counts vary significantly with age Red blood cells Physiologic anemia • Hemoglobin • White blood cells

Anemia A reduction of the red blood cell mass or hemoglobin concentration below the range of values occurring in healthy persons 回复旦大学上海医学院儿科学系

Anemia A reduction of the red blood cell mass or hemoglobin concentration below the range of values occurring in healthy persons

Lower limit of normal hemoglobin values at various ages neonate Hb<1459/L Hb<90g儿L 4~6m Hb 100 a/L 6m w by Hb< 110 g/L 6y~14yHb<1209/L Degree of anemia Mild Moderate Severe Extremely severe ~90q儿L~60q/L ~30q/L <309/L Neonate w120 g/L M90 g/L 60 g/L <60q/L 回复旦大学上海医学院儿科学系

Lower limit of normal hemoglobin values at various ages neonate Hb  145 g/L  1 ~ 4m Hb  90 g/L  4 ~ 6m Hb  100 g/L 6m ~ 6y Hb  110 g/L 6y ~ 14y Hb  120 g/L Degree of anemia Mild Moderate Severe Extremely severe ~90 g/L ~60 g/L ~30 g/L  30 g/L Neonate ~120 g/L ~90 g/L ~60 g/L  60 g/L

Y Anemia is not a specific entity but an indication of an underlying pathologic process or disease v Two useful classification of anemias Physiologic(erythrokinetic) classification 1. those resulting primarily from decreased production of red blood cells or hemoglobin 2. those in which increased destruction or loss of red blood cells Morphologic classification the red blood cells being characterized by their mean corpuscular volume(MCV) as microcytic(MCV 100f ), or normocytic (75-100fl) 回复旦大学上海医学院儿科学系

✓ Anemia is not a specific entity but an indication of an underlying pathologic process or disease ✓ Two useful classification of anemias Physiologic (erythrokinetic) classification 1. those resulting primarily from decreased production of red blood cells or hemoglobin 2. those in which increased destruction or loss of red blood cells Morphologic classification the red blood cells being characterized by their mean corpuscular volume (MCV) as microcytic (MCV 100fl), or normocytic (75-100fl)

Classification of the Anemias Anemias resulting primary from inadequate production of red blood cells or hemoglobin Hemolytic anemias EXtrinsic(extracellular) abnormalities Nonimmunologic disorders 回复旦大学上海医学院儿科学系

Classification of the Anemias • Anemias resulting primary from inadequate production of red blood cells or hemoglobin • Hemolytic anemias • Extrinsic (extracelllular) abnormalities • Nonimmunologic disorders

Anemias resulting primary from inadequate production of red blood cells or hemoglobin Decreased numbers of red blood cell precursors in the marrow" Pure red blood celpanemia Congenital pure red blood cell anemia Acquired pure red blood cell anemias(e. g. TEC) Inadequate production despite normal numbers of red blood cell precursors Anemia of infection, inflammation, and cancer Anemia of chronic renal disease Congenital dyserythropoietic anemias 回复旦大学上海医学院儿科学系

Anemias resulting primary from inadequate production of red blood cells or hemoglobin Decreased numbers of red blood cell precursors in the marrow “Pure red blood cell” anemia Congenital pure red blood cell anemia Acquired pure red blood cell anemias (e.g. TEC) Inadequate production despite normal numbers of red blood cell precursors Anemia of infection, inflammation, and cancer Anemia of chronic renal disease Congenital dyserythropoietic anemias

Anemias resulting primary from inadequate production of red blood cells or hemoglobin Deficiency of specific factors Megaloblastic anemias Folic acid deficiency or malabsorption Vitamin B12 deficiency, malabsorption, or transport Orotic aciduria Microcytic anemias Iron deficiency Pyridoxine-responsive and X-linked hypochromic anemias Lead poisoning Copper deficiency Thalassemia trait (國)复旦大学上海医学院儿科学系

Anemias resulting primary from inadequate production of red blood cells or hemoglobin Deficiency of specific factors Megaloblastic anemias Folic acid deficiency or malabsorption Vitamin B12 deficiency, malabsorption, or transport Orotic aciduria Microcytic anemias Iron deficiency Pyridoxine-responsive and X-linked hypochromic anemias Lead poisoning Copper deficiency Thalassemia trait