大四下兒科12級(jí)紙質(zhì)檔08.1blood cell counts and anemia48

1、BLOOD DISORDERSDr.Jie Yu, MD. ProfessorThe department of Pediatric, Hematology/OncologyHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALContentsContentsn Blood cell countsn Anemian Iron Deficiecny AnemiaHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALWhat do you need to know about pediatric blood disorders? HEMATOLOGY

2、/ONCOLOGY, CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL Development of Development of Hematopoietic OrgansHematopoietic OrgansFetal HematopoiesisliverBlood Cell Counts Blood Cell Counts and Hemoglobin and Hemoglobin HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALRBC and Hb LevelRBC and Hb Level

3、 At Birth: RBC: 5-7x1012/L Hb: 150 to 230g/L. HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALPolycythemia紅血球增多癥紅血球增多癥 AgeAnemiaNewborn 145g/L1-4 mo 90g/L4-6 mo 100g/L6 mo-6 yr 110g/L6-14 yr 120g/LRBC and Hb LevelRBC and Hb Level At Birth: physiological anemia / Postnatal fall Hemoglobin values in term infan

4、ts drop to their lowest mean of 100g/L at 2-3 mo Preterm infantHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALAgeAnemiaNewborn 145g/L1-3 mo 90g/L4-6 mo 100g/L6 mo-6 yr 110g/L6-14 yr 120g/LRBC and Hb levelRBC and Hb level Physiological Anemia. Causes Erythropoietin production Red cell life span (90/120) Bloo

5、d volume HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALRBC and Hb LevelRBC and Hb Level At Birth: physiology anemia / Postnatal fall Infancy Preschool age: RBC: 4 x 1012/L Hb: 110 g/L 7-12yr: adult level Hb: 120 g/LHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALAgeAnemiaNewborn 145g/L1-4 mo 90g/L4-6 mo 100g/L6 mo-

6、6 yr 110g/L6-14 yr 120g/LRBC 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 level Reticulocytes（網(wǎng)織紅細(xì)胞）（網(wǎng)

7、織紅細(xì)胞） 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 HOSPITALRBC and Hb levelRBC and Hb level Reticulocytes（網(wǎng)織紅細(xì)胞）（網(wǎng)織紅細(xì)胞）and nucleated red blood cells Persistence of a

8、 high reticulocyte count is abnormal and often suggests a hemolytic process or blood loss. Nucleated RBC present in peripheral blood often suggests active hematopoiesis respond to hemolysis or blood lossHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL HEMOGLOBIN 血紅蛋白血紅蛋白. HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPI

9、TAL Function transport oxygen. Construction iron-containing heme plus globins which is a tetramer made up of two pairs of polypeptide chains, 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,

10、CHILDRENS HOSPITAL HEMOGLOBIN. HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALQA: 2歲后血紅蛋白組分及比例？歲后血紅蛋白組分及比例？ Hb A (22) : 95% Hb A2 (2 2): 3.5% Hb F (2 2): 2.0%WBC CountsWBC Counts At birth: 20 x 109/L Infant: 12 x 109/L Preschool: 8.0 x 109/LHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL (%)70淋巴細(xì)胞淋巴細(xì)胞中性粒細(xì)胞中性粒細(xì)胞4-6歲

11、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 HOSPITALWBC CountsWBC Counts 簡(jiǎn)單病例簡(jiǎn)單病例 男孩，男孩，2歲歲2月，因發(fā)熱月，因發(fā)熱1天就診天就診 查體發(fā)現(xiàn)：查體發(fā)現(xiàn)：T38,面色紅潤(rùn)，咽部面色紅潤(rùn)，咽部充血，扁桃充血，扁桃1度，肺心（度，肺心（-） WBC 10 x 109/L, N 0.30, L 0.68 ? WBC 18 x 109/L, N 0.70, L 0.28

12、?HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALPLT & Blood VolumePLT & Blood Volume PLT: 100-300 x 109/L Blood Volume: Term newborn: 85ml/kg Premature infant: 95ml/kg Adult: 75ml/kg Young children: 75-80ml/kgHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALThe Introduction of The Introduction of ANEMIAANEMIA

13、HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALPrevalence of anemia in children 0-5 years old WHO region, 1998-2005Definition 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 是指單位容積內(nèi)的紅細(xì)胞數(shù)、血紅蛋白是指單位容積內(nèi)的紅細(xì)

14、胞數(shù)、血紅蛋白量或紅細(xì)胞比容低于正常值。量或紅細(xì)胞比容低于正常值。HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALTable 4. The definition of AnemiaTable 4. The definition of Anemia年齡年齡貧血值貧血值neonate 145g/L1-4月月 90g/L4-6月月 100g/L6月月-6歲歲 110g/L6-14歲歲 120g/LHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALTable 4. T

15、he degree of AnemiaTable 4. The degree of AnemiaMild Moderate Severe Extremely children-90g/L-60g/L-30g/L30g/Lneonate144-120g/L-90g/L-60g/L94 32 32-38Normocromic /Normocytic 80-94 28-32 32-38Microcytic 80 28 32-28Hypochromic /Microcytic 80 28 32 Classification-morphologyClassification-morphologyTabl

16、e 5HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification- Etiology Classification- Etiology HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALReduced capacity to produce red blood cells紅細(xì)胞生成減少紅細(xì)胞生成減少Hemolysis紅細(xì)胞破壞紅細(xì)胞破壞溶血性貧血溶血性貧血Blood Loss失血性貧血失血性貧血Classification- etiology Classification- etiology Aplastic anem

17、ia 再生障礙性貧血再生障礙性貧血 Fanconis anemia Acquired aplastic anemia Pure red cell aplasia 純紅細(xì)胞再生障礙純紅細(xì)胞再生障礙性貧血性貧血 congenital hypoplastic anemia (Diamond-Blackfan) Acquired hypoplastic anemiaHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALl Reduced capacity to produce red blood cellsClassification- etiology Classificat

18、ion- etiology Aplastic anemia 再生障礙性貧血再生障礙性貧血原發(fā)性或獲得性原發(fā)性或獲得性貧血、感染、出血的表現(xiàn)貧血、感染、出血的表現(xiàn)脾臟不腫大脾臟不腫大外周血三系減低，網(wǎng)織紅細(xì)胞減低外周血三系減低，網(wǎng)織紅細(xì)胞減低骨髓病理幫助確診骨髓病理幫助確診免疫抑制劑治療免疫抑制劑治療+造血干細(xì)胞移植造血干細(xì)胞移植HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALClassification- etiologyClassification- etiologyMarrow Infiltration 骨髓浸潤(rùn)骨髓浸潤(rùn)Leukemia 白血病白血病Lymph

19、oma 淋巴瘤淋巴瘤Neuroblastom 神經(jīng)母細(xì)胞瘤神經(jīng)母細(xì)胞瘤LCH 朗格罕細(xì)胞組織細(xì)胞增生癥朗格罕細(xì)胞組織細(xì)胞增生癥HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALl Reduced capacity to produce red blood cellsClassification- etiology Classification- etiology Deficiency Syndrome Iron Folate Vitamin B12 Vitamin E Vitamin B6HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITAL

20、l Reduced capacity to produce red blood cellsClassification- etiologyClassification- etiology Iintrinsic RBC abnormalities Hemoglobinopathies Enzymopathies Membrane disorders extrinsic RBC abnormalities Immunologic: AIHA othersHEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALl HemolysisClassification- etiolog

21、y Classification- etiology Intrinsic membrane defects Hereditary Spherocytosis: （遺傳性球形紅細(xì)胞增多癥）（遺傳性球形紅細(xì)胞增多癥） Hemoglobinopathy Thalassemia （地中海貧血）（地中海貧血） RBC enzyme defects G6PD deficiency （紅細(xì)胞葡萄糖（紅細(xì)胞葡萄糖-6-磷酸脫氫酶缺乏癥）磷酸脫氫酶缺乏癥）HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALl Hemolysis: intrinsic RBC abnormalities

22、Classification- etiologyClassification- etiology Gastrointestinal bleeding Ucler 消化道潰瘍消化道潰瘍 Ankylostomiasis （鉤蟲?。ㄣ^蟲病） Menstrual （月經(jīng)）（月經(jīng)） Trauma （外傷）（外傷）HEMATOLOGY/ONCOLOGY, CHILDRENS HOSPITALl Blood LossHYPOCHROMICMICROCYTICNORMOCHROMIC NORMOCYTICMACROCYTICRETICULOCYTE COUNTIron deficiency-Chronic

23、blood loss-Poor dietary intake-Cows milk protein intoleranceThalassemiaChronic inflammatory diseaseLead intoxicationCopper deficiencyChronic inflammatory diseaseMalignancy marrow infitritionRecent blood lossUremiaAplasia/ hypoplasiVitaminB12 deficiencyFolate deficiencyChronic liver diseaseMarrow failure-Fanconi anemia-Aplastic anemia-MyelodysplasiaDrugs -AxidothymidineHEMOLYSISHemoglobinopathy-thalassemiaEnzymopathy-G6PD deficiencyMembranopa