兒童造血系統(tǒng)和貧血簡介-Introduction-of-Hematopoietic-System-and-Anemia-in-Children課件

1、 HU QUN Department of pediatrics Tongji hospitalIntroduction of Hematopoietic System and Anemia in Children1 HU QUN Introduction of 2 months girl，Hb100g/L，normal？one year old boy，WBC10109/L，N60%，L40%，normal？The question from parents22 months girl，Hb100g/L，normal？1 hour new born，vomiting blood，is blo

2、od from mother or from itself？The question from doctor3The question from doctor3Hematopoiesis ：Hematopoiesis in fetus Hematopoiesis after birth Blood features：RBC and HbWBCBPCBlood volume4Hematopoiesis ：4Hematopoiesis in fetusDevelopmental hematopoiesis occurs in three anatomic stages: mesoblastic（y

3、olk sac）hepatic（liver）myeloid（marrow）5Hematopoiesis in fetusDevelopmHematopoiesis in fetus6Hematopoiesis in fetus6Hematopoiesis after birth Active hematopoietic tissue (red marrow) Fatty tissue (yellow marrow)1. myeloid hematopoiesisThe yellow marrow can change to red marrow in response to certain s

4、evere hematologic stresses7Hematopoiesis after birth Actimyeloid hematopoiesisinfancy57yr18yr8myeloid hematopoiesisinfancy5 Infant: all bone was full of red marrow, no yellow marrow, no hematopoiesis store young children: more red marrow, less yellow marrow, a little hematopoiesis store Older childr

5、en and adult: less red marrow, more yellow marrow, more hematopoiesis store9 Infant: all bone was full of in infant, Liver, splen may be a hematopoiesis organ in response to certain severe hematologic stresses (hemolytic anemia, infection, etc), just like the fetus. 2、extramedullary hematopoiesisIt

6、will return to the normal after treatment10 in infant, Blood featuresRBC and HbHb ：from highlow normalRBC： from highlow normal11Blood featuresRBC and Hb11physiological anemiacause：RBC production，RBC destruction，blood dilutiontime：23 monthIn term infant : Hb100g/L in preterm infant: Hb 7090g/L12physi

7、ological anemiacause：RBC 1.WBC： from high to low to nomal（at 8 years old） (10109/L in infancy)WBC13WBC132.The change of WBCin childrenNL10.54-6days4-6yr142.The change of WBCin childreBPC：150109250109/LBPC15BPC15At birth： HbF 70% HbA 30% HbA2 1%1 yr ： HbF5% HbA 95% HbA2 2%3%More than 2 yr ： HbF2% HbA

8、 95% HbA2 2%3% The type of Hb16At birth： HbF 70% Hblood volumeNewborn： 10% bodyweightChildren：8%10% bodyweightAdult：6%8% bodyweightIt is easy to get dehydration in children17blood volumeNewborn： 10% bodyw2 months girl，Hb100g/L，normal？one year old boy，WBC10109/L，N60%，L40%，normal？The question from par

9、ents182 months girl，Hb100g/L，normal？1 hour new born，vomiting blood，is blood from mother or from itself？The question from doctor191 hour new born，vomiting bloodAnemia in childrenAnemia in children20Anemia in childrenAnemia in chWhat is anemia：Hb/RBC is lower than normal21What is anemia：216 month： new

10、 born：Hb145g/L 26 month :Hb100g/L6month6y： Hb110g/L6y14y： Hb120g/L What is normal?22What is normal?221.Classification from cause of anemiaHypoproduction disorderHemorrhage disease (blood loss)Hemolytic disorder231.Classification from cause ofCause of anemia24Cause of anemia242.Classification from mo

11、rphology：MCV（fl）MCH（pg）MCHC（g/L）normal80942832320380Normocytic,normochromic80942832320380Macrocytic9432320380Microcytic8028320380Microcytic,hypochronic8028320252.Classification from morpholoClassification from RBC morphology Microcytic, hypochromic anemia1.Defects of haem synthesis IDA Chronic infla

12、mmation2.Defects of globin synthesis thalassemia26Classification from RBC morphoNormocytic, normochromic anemia1.hemolytic anemia Intrinsic red cell defectsMembrane defects: spherocytosisHemoglobinopathies: thalassemiaEnzymopathies: G6PD deficiency Extrinsic disordersImmune-mediated: Rh incompatibil

13、ityHypersplenism27Normocytic, normochromic anemi2.Hemorrhage (acute or chronic) Hookworm infection Meckels diverticulum Menstruation3.hypoproduction disorders Red cell aplasia: renal disease Pancytopenia: AA, leukemia282.Hemorrhage (acute or chronicMacrocytic anemia1.bone marrow megaloblasticVit B12

14、 deficiencyFolic acid deficiency2.bone marrow not megaloblasticHypothyroidismFanconi anemia29Macrocytic anemia29Clinical featuresPallorHepatosplenomegalyOthers30Clinical featuresPallor30weakness, tachypnea, shortness of breath on exertion, tachycardia, cardiac dilatation, and congestive heart failur

15、e31weakness, tachypnea, shortnessDiagnosis Is anemia associated with other hematologic abnormalities? If yes, consider:Aplastic anemiaLeukemiaOther bone marrow replacement disorders32Diagnosis Is anemia associatIs anemia associated with reticulocytosis? If yes, usually a consequence of bleeding or o

16、ngoing hemolysis33Is anemia associated with reti Is there associated hyperbilirubinemia or increased serum lactate dehydrogenase If yes, usually due to hemolysis34 Is there associated hyperbiReview of peripheral blood smearSpherocytes (hereditary spherocytosis, autoimmune hemolytic anemia)Sickle for

17、ms (sickle cell disease)Target cells (-thalassemia)Microangiopathy (hemolytic-uremic syndrome, thrombotic thrombocytopenia35Review of peripheral blood smeIs anemia associated with a lower than appropriate reticulocyte response? If yes, assess red blood cell size36Is anemia associated with a loAre re

18、d blood cells microcytic? If yes, usually due to defect in hemoglobin synthesisIron deficiency or -Thalassemia Lead poisons37Are red blood cells microcyticAre red blood cells macrocytic? If yes, is there neutrophil hypersegmentation (megaloblastic changes)?If yes, considerFolate deficiency, vitamin B12 deficiency, inborn errors of meta