兒科學(xué)教學(xué)課件：Approach to Childhood Anemia

ApproachtoChildhoodAnemiaImportantto

RememberonAnemiaAnemiaisaclinicalsignofdisease.Itisnotasinglediseasebyitself.Needtolookfortheunderlyingcause!Willweignoreafeverwithoutinvestigation?DrugAdministrationdependsonthecause.

extramedullaryhematopoiesisSitesoferythropoiesis:extramedullaryhematopoiesis

MorphologyofnormalredbloodcellsNormalredbloodcell(erythrocyte)issmall,round,andbiconcaveMeancorpuscularvolume, MCV80-94flMeancorpuscularhemoglobin, MCH28-32pgMeancorpuscularhemoglobinconcentration,MCHC32-38%Howtoworkupananemicpatient?History:age,duration,diet,complaintPE:jaundice,hepatomegaly,thrillCBC:MCV,MCH,MCHCRet&RPI(Reticulocyteproductionindex):RPI=[Ret(%)×(PatientHct)/(NormalHct)]/2Peripheralsmearexamination1yroldboy:Hb7.8g/dL;reticulocytecount4.2%;MCV60.4fl,MCH24.6pg/L,MCHC30.5%.RPI???1ststep:Anemia?MeasurementNormalRangeRBCcount5million/mm34.0~5.7Hemoglobin(Hb)15g/dL12~17Hematocrit(Hct)45%38~50Ax3=Bx3=C-Thisistheruleofthumb

NormalchildrenThechangeofRBCandHb

birth10d2～3m12y

(age)

654321(180)(150)(120)(90)(60)(30)HbRBC654321

AgeHb(g/L)Atbirth1451-4m904-6m1006m-6y1106-14y120TheDiagnosticCriteria2ndstep:

Reticulocyteproductionindex=?3rdstep:SizeofRBC?

Irondeficiency(IDA)ThalassemiasLeadpoisonSideroblasticAnemiaChronicinfectionsBonemarrowdisorder:AplasticanemiaTumorinfiltrationMDSMegaloblasticanemia:VitB12FolicacidDifferentiationofmicrocyticanemiaIDAThalassemiasSideroblasticAnemiaChronicdiseaseserumironN,serumferritinN,HbelectrophoresisNHbF/HbA2NNhemolysisimmunenon-immuneCoombs(+)Coombs(-)AIHA

allogenicRPI>2ABO,Rhtransfusion4thstep:immune-related?PrimarySecondarynon-immunehemolysismicroangiopathicmembranedefects(Hereditaryspherocytosis)DIC/TTP/HUSmechanicalheartvalvecongenitalPosterioritysnakevenomsUnstablehemoglobins(Thalassemias)Enzymedeficiencies(G-6-PD)DiagnosisEvaluationHemoglobinopathyHbelectrophoresisMembranedisorderOsmoticfragility,

MorphologyEnzymedeficienciesPK/G6PDscreen,reductionofmethemoglobinAutoimmuneCoombstestMicroangiopathicMorphology,History,BUN/CrPNHHamtest,flowcytometryDifferentiationofHemolyticanemiaLastbutnotleast-HemorrhagePepticulcerHiatusherniaMeckeldiverticulumIntestinalduplicationParasiticinfectionPulmonaryhemorrhageidiopathicpulmonaryhemosiderosisApproachtoanemia

CasestudyAneightmonthsoldgirlreferredtotheclinicduetoprogressingpalefor2months.Thepatienthadnofever,norash,nocough,novomiting,nodiarrhea,nosleepiness,andnocramp.Shetooknomedication.Sinceonset,herappetiteisgood,andbothherspiritednessandphysicalenergyarenormal.Defecationandurinationarenormal.Pasthistory：Nohistoryofseverdisease,operation,traumaandallergytodrugs(foods).Personalhistory：G1P1,35weeksprematureinfant,naturalbirth,thebirthweightis2.5kilograms,noasphyxiationatbirth,breastfeeding,normaldevelopment,historyofpreventiveimmunizationisnormal.Familyhistory：herparentsareingoodhealthy.Noconsanguineousmarriage.Nofamilyhistoryofhepatitis,TBandotherinfectiousdiseases.Nohistoryofhemorrhagicdisordersandanemia.Physicalexamination:Alert,ingoodspirits.T36.4°C,P124times/min，R26，BP84/63mmHg，W12.5KG，H67cmAnemicpalefacies,skinandmucosalookpale.Theskinandscleraarenotstainedyellow.Superficiallymphnodesarenotenlarged.Heartandlungsshownoabnormalities.Abdomenisflatandsoft.Liverisnotreached.Spleenisjustpalpatedundertheleftribedge.Nocyanosis.Nopigmentation.Noskineruption.Extremities:noarticularswelling,norashorlesions,freemovementsofalllimbs.Physiologicalreflexesareexistentwithoutanypathologicalones.Laboratoryexamination:Completebloodcount:WBC9.7×109/L,L62.4%,N32.2%;RBC2.8×1012/L,Hb65g/L,MCV59.7fl,MCH18.1pg,MCHC30%,Ret2.3%,CRP:<1mg/L,StoolOB:(-),UrineRt:(-);Biochemicalexamination:T-Bili20.2μmol/L,D-Bili6.9μmol/L,I-Bili13.3μmol/L,LDH264U/L.

PossibleDiagnosis?Differentiation?1ststep:Anemia?AgeHb(g/L)Atbirth1451-4m904-6m1006m-6y1106-14y1202ndstep:

Reticulocyteproductionindex=?3rdstep:SizeofRBC?DifferentiationofmicrocytichypochromicalanemiaIrondeficiencyanemiaNutritional Chronicbloodloss:GIlossorrespiratorytractlossauchasIPHThalassemiaSideroblasticanemiaLeadPoisoningChronicinfectionsFurtherexaminations?Furtherexamination:Serumferritinassay：3.4ng/ml(normalrange11.0-306.8ng/ml)RedCellMorphology:variabilityofredcellsize,majoritymicrocyte,centrallight-stainedregionenlarged.Osmoticfragilitytestoferythrocytes：Atthebeginningofhemolysis0.46%（control0.44%）Completehemolysis0.28%（control0.32%）Hbelectrophoresis：HbA96.7%,HbF1.9%,HbA21.4%Methemoglobinreductiontest：85%（Normal>75%）Coombstest：negativeBonemarrow:Granulocyticproliferationisreduced(32.5%),majoritymetamyelocyteandbandgranulocyte,morphologyisnormal.Erythroidhyperplasia(40.5%),majorityinmedianandlateimmaturestage,variabilityofredcellsize,fragmentedcytoplasmwithpoorhemoglobinizationIronstaining:intracellular3%(normal>15%),