医学精品课件：08.1blood cell counts and anemia

BLOODDISORDERSDr.JieYu,MD.ProfessorThedepartmentofPediatric,Hematology/OncologyHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALContentsBloodcellcountsAnemiaIronDeficiecnyAnemiaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALWhatdoyouneedtoknowaboutpediatricblooddisorders?HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

Developmentof

HematopoieticOrgansFetalHematopoiesisliverBloodCellCounts

andHemoglobin

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALRBCandHbLevelAtBirth:

RBC:5-7x1012/LHb:150to230g/L.

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALPolycythemia红血球增多症

AgeAnemiaNewborn<145g/L1-4mo<90g/L4-6mo<100g/L6mo-6yr<110g/L6-14yr<120g/LRBCandHbLevelAtBirth:

physiologicalanemia/PostnatalfallHemoglobinvaluesinterminfantsdroptotheirlowestmeanof100g/Lat2-3moPreterminfantHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALAgeAnemiaNewborn<145g/L1-3mo<90g/L4-6mo<100g/L6mo-6yr<110g/L6-14yr<120g/LRBCandHblevelPhysiologicalAnemia.

CausesErythropoietinproductionRedcelllifespan(90/120)Bloodvolume

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALRBCandHbLevel

AtBirth:

physiologyanemia/PostnatalfallInfancyPreschoolage:RBC:4x1012/LHb:110g/L7-12yr:adultlevelHb:120g/LHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALAgeAnemiaNewborn<145g/L1-4mo<90g/L4-6mo<100g/L6mo-6yr<110g/L6-14yr<120g/LRBCandHbLevelAtBirth:

RBC:5-7x1012/LHb:150to230g/L.

Postnatalfall/physiologyanemiaInfancyPreschoolage:RBC:4x1012/LHb:110g/L7-12yr:adultlevelHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALRBCandHblevelReticulocytes（网织红细胞）AtBirth:5%/10%1-2mo:falldownto0.3%Lateradultlevel:0.5-1.5%NucleatedRedBloodCellsAtbirth:3-10/100WBC;10-20/100WBC1wk:disappearHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALRBCandHblevelReticulocytes（网织红细胞）andnucleatedredbloodcellsPersistenceofahighreticulocytecountisabnormalandoftensuggestsahemolyticprocessorbloodloss.NucleatedRBCpresentinperipheralbloodoftensuggestsactivehematopoiesisrespondtohemolysisorbloodlossHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

HEMOGLOBIN血红蛋白.

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALFunction

transportoxygen.

Constructioniron-containinghemeplusglobinswhichisatetramermadeupoftwopairsofpolypeptidechains,

Table3.HEMOGLOBINS

HbChains8周6月出生6-12月2岁EmbryGower1ζ2ε28周前，3月消失

Gower2α2ε2

Portlanζ2γ2

FetalHbFα2γ2

增加90%70%<5%<2%AdultHbAα2β2

5-10%30%>95%

HbA2α2δ2

<1%2-3%<3.5%>95%HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

HEMOGLOBIN.

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALQA:2岁后血红蛋白组分及比例？

HbA(α2β2):>95%HbA2(α2δ2):<3.5%HbF(α2γ2):<2.0%WBCCountsAtbirth:20x109/LInfant:12x109/LPreschool:8.0x109/LHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

(%)70淋巴细胞中性粒细胞4-6岁4-6天60

50

40

30

20

1013579

日数1357911

岁数

Fig4.WBCRatio

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALWBCCounts简单病例男孩，2岁2月，因发热1天就诊查体发现：T38℃,面色红润，咽部充血，扁桃1度，肺心（-）WBC10x109/L,N0.30,L0.68?WBC18x109/L,N0.70,L0.28?HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALPLT&BloodVolumePLT:

100-300x109/LBloodVolume:Termnewborn:85ml/kgPrematureinfant:95ml/kgAdult:75ml/kgYoungchildren:75-80ml/kgHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALTheIntroductionofANEMIAHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALPrevalenceofanemiainchildren

0-5yearsoldWHOregion,1998-2005DefinitionofAnemia

Areductionoftheredbloodcellvolumeorhemoglobinconcentrationbelowtherangeofvaluesoccurringinhealthypersons是指单位容积内的红细胞数、血红蛋白量或红细胞比容低于正常值。HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALTable4.ThedefinitionofAnemia年龄贫血值neonate<145g/L1-4月<90g/L4-6月<100g/L6月-6岁<110g/L6-14岁<120g/LHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALTable4.ThedegreeofAnemiaMildModerateSevereExtremelychildren-90g/L-60g/L-30g/L<30g/Lneonate144-120g/L-90g/L-60g/L<60g/LManifestationofAnemia一般表现皮肤黏膜苍白造血器官肝脏、脾脏、淋巴结肿大外周血有核红细胞、幼稚粒细胞系统表现循环呼吸：呼吸心率加快、心脏扩大、收缩期杂音消化系统：食欲减低、恶心、腹胀、便秘神经系统：嗜睡烦躁、注意力不集中、头晕、耳鸣；智力减退、神经精神发育障碍HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALClassificationofAnemiaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL形态学分类morphology

病因学分类etiology

MCV（fl）MCH（pg）MCHC（%）Normalranges80-9428-3232-38Macrocytic>94>3232-38Normocromic/Normocytic80-9428-3232-38Microcytic

<80<2832-28Hypochromic/Microcytic

<80<28<32Classification-morphologyTable5HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALClassification-Etiology

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALReducedcapacitytoproduceredbloodcells红细胞生成减少Hemolysis红细胞破坏—溶血性贫血BloodLoss失血性贫血Classification-etiology

Aplasticanemia再生障碍性贫血Fanconi’sanemiaAcquiredaplasticanemiaPureredcellaplasia

纯红细胞再生障碍性贫血congenitalhypoplasticanemia (Diamond-Blackfan)AcquiredhypoplasticanemiaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALReducedcapacitytoproduceredbloodcellsClassification-etiology

Aplasticanemia再生障碍性贫血原发性或获得性贫血、感染、出血的表现脾脏不肿大外周血三系减低，网织红细胞减低骨髓病理帮助确诊免疫抑制剂治疗+造血干细胞移植HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALClassification-etiologyMarrowInfiltration骨髓浸润Leukemia白血病Lymphoma淋巴瘤Neuroblastom

神经母细胞瘤LCH朗格罕细胞组织细胞增生症HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALReducedcapacitytoproduceredbloodcellsClassification-etiology

DeficiencySyndromeIronFolateVitaminB12VitaminEVitaminB6HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALReducedcapacitytoproduceredbloodcellsClassification-etiologyIintrinsicRBCabnormalitiesHemoglobinopathiesEnzymopathiesMembranedisordersextrinsicRBC

abnormalitiesImmunologic:AIHAothersHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

HemolysisClassification-etiology

IntrinsicmembranedefectsHereditarySpherocytosis:

（遗传性球形红细胞增多症）HemoglobinopathyThalassemia

（地中海贫血）RBCenzymedefectsG6PDdeficiency

（红细胞葡萄糖-6-磷酸脱氢酶缺乏症）HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

Hemolysis:intrinsicRBCabnormalitiesClassification-etiologyGastrointestinalbleedingUcler

消化道溃疡Ankylostomiasis

（钩虫病）Menstrual（月经）Trauma（外伤）HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALBloodLossHYPOCHROMICMICROCYTICNORMOCHROMICNORMOCYTICMACROCYTICRETICULOCYTECOUNTIrondeficiency-Chronicbloodloss-Poordietaryintake-Cow’smilkproteinintoleranceThalassemiaChronicinflammatorydiseaseLeadintoxicationCopperdeficiencyChronicinflammatorydiseaseMalignancymarrowinfitritionRecentbloodlossUremiaAplasia/hypoplasiVitaminB12deficiencyFolatedeficiencyChronicliverdiseaseMarrowfailure-Fanconianemia-Aplasticanemia-MyelodysplasiaDrugs-AxidothymidineHEMOLYSISHemoglobinopathy-thalassemiaEnzym