醫(yī)學(xué)精品課件：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紅血球增多癥

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（網(wǎng)織紅細胞）AtBirth:5%/10%1-2mo:falldownto0.3%Lateradultlevel:0.5-1.5%NucleatedRedBloodCellsAtbirth:3-10/100WBC;10-20/100WBC1wk:disappearHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALRBCandHblevelReticulocytes（網(wǎng)織紅細胞）andnucleatedredbloodcellsPersistenceofahighreticulocytecountisabnormalandoftensuggestsahemolyticprocessorbloodloss.NucleatedRBCpresentinperipheralbloodoftensuggestsactivehematopoiesisrespondtohemolysisorbloodlossHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

HEMOGLOBIN血紅蛋白.

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALFunction

transportoxygen.

Constructioniron-containinghemeplusglobinswhichisatetramermadeupoftwopairsofpolypeptidechains,

Table3.HEMOGLOBINS

HbChains8周6月出生6-12月2歲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歲后血紅蛋白組分及比例？

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

(%)70淋巴細胞中性粒細胞4-6歲4-6天60

50

40

30

20

1013579

日數(shù)1357911

歲數(shù)

Fig4.WBCRatio

HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALWBCCounts簡單病例男孩，2歲2月，因發(fā)熱1天就診查體發(fā)現(xiàn)：T38℃,面色紅潤，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是指單位容積內(nèi)的紅細胞數(shù)、血紅蛋白量或紅細胞比容低于正常值。HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALTable4.ThedefinitionofAnemia年齡貧血值neonate<145g/L1-4月<90g/L4-6月<100g/L6月-6歲<110g/L6-14歲<120g/LHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALTable4.ThedegreeofAnemiaMildModerateSevereExtremelychildren-90g/L-60g/L-30g/L<30g/Lneonate144-120g/L-90g/L-60g/L<60g/LManifestationofAnemia一般表現(xiàn)皮膚黏膜蒼白造血器官肝臟、脾臟、淋巴結(jié)腫大外周血有核紅細胞、幼稚粒細胞系統(tǒng)表現(xiàn)循環(huán)呼吸：呼吸心率加快、心臟擴大、收縮期雜音消化系統(tǒng)：食欲減低、惡心、腹脹、便秘神經(jīng)系統(tǒng)：嗜睡煩躁、注意力不集中、頭暈、耳鳴；智力減退、神經(jīng)精神發(fā)育障礙HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALClassificationofAnemiaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL形態(tài)學(xué)分類morphology

病因?qū)W分類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紅細胞生成減少Hemolysis紅細胞破壞—溶血性貧血BloodLoss失血性貧血Classification-etiology

Aplasticanemia再生障礙性貧血Fanconi’sanemiaAcquiredaplasticanemiaPureredcellaplasia

純紅細胞再生障礙性貧血congenitalhypoplasticanemia (Diamond-Blackfan)AcquiredhypoplasticanemiaHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALReducedcapacitytoproduceredbloodcellsClassification-etiology

Aplasticanemia再生障礙性貧血原發(fā)性或獲得性貧血、感染、出血的表現(xiàn)脾臟不腫大外周血三系減低，網(wǎng)織紅細胞減低骨髓病理幫助確診免疫抑制劑治療+造血干細胞移植HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALClassification-etiologyMarrowInfiltration骨髓浸潤Leukemia白血病Lymphoma淋巴瘤Neuroblastom

神經(jīng)母細胞瘤LCH朗格罕細胞組織細胞增生癥HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALReducedcapacitytoproduceredbloodcellsClassification-etiology

DeficiencySyndromeIronFolateVitaminB12VitaminEVitaminB6HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALReducedcapacitytoproduceredbloodcellsClassification-etiologyIintrinsicRBCabnormalitiesHemoglobinopathiesEnzymopathiesMembranedisordersextrinsicRBC

abnormalitiesImmunologic:AIHAothersHEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

HemolysisClassification-etiology

IntrinsicmembranedefectsHereditarySpherocytosis:

（遺傳性球形紅細胞增多癥）HemoglobinopathyThalassemia

（地中海貧血）RBCenzymedefectsG6PDdeficiency

（紅細胞葡萄糖-6-磷酸脫氫酶缺乏癥）HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITAL

Hemolysis:intrinsicRBCabnormalitiesClassification-etiologyGastrointestinalbleedingUcler

消化道潰瘍Ankylostomiasis

（鉤蟲病）Menstrual（月經(jīng)）Trauma（外傷）HEMATOLOGY/ONCOLOGY,CHILDREN’SHOSPITALBloodLossHYPOCHROMICMICROCYTICNORMOCHROMICNORMOCYTICMACROCYTICRETICULOCYTECOUNTIrondeficiency-Chronicbloodloss-Poordietaryintake-Cow’smilkproteinintoleranceThalassemiaChronicinflammatorydiseaseLeadintoxicationCopperdeficiencyChronicinflammatorydiseaseMalignancymarrowinfitritionRecentbloodlossUremiaAplasia/hypoplasiVitaminB12deficiencyFolatedeficiencyChronicliverdiseaseMarrowfailure-Fanconianemia-Aplasticanemia-MyelodysplasiaDrugs-AxidothymidineHEMOLYSISHemoglobinopathy-thalassemiaEnzym