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1、Diseaseofhematopoieticsysteminfantileanemia（1）nutritionalirondeficiencyanemia(IDA)（2）nutritionalmegaloblasticanemiaPrimary/immunitythrombocytopeniaPurpura(ITP)Leukemiahaematogenesisofchildrenhematopoiesis-producedbloodextramedullarybeforebirthandpostnatalmesoblasthepaticmedullary3-15w6w-6ms3msEmbryo
2、stageMesoblastichaematogenesis：3wsbegin，8wsweaken,12-15wsdisappears。liver：8wsbegin，6monthsgraduallyweaken,erythroblast、granularcellandmegakaryocyte.Embryostage3、spleen：12wsbeginerythrocyte,granule,lymphocyte4、Haematogenesisoflymphaticorgan1.thoracicgland:8ws2.lymphaticnodes:11wsEmbryostage5、myelo-ha
3、ematopoiesis：6monsHaematogenesisfunctionemphasis，makevariouskindsofbloodcells，uniquehematogenicorgan afterbirth.Haematopoiesispostnatal1、marrow：2、extramedullary：whenrequirementofhaemopoiesisincrease，liver、spleen、lymphadenectasis，hepatomegalyandsplenomegaly,incirculatingbloodimmatureerythrocytesandgr
4、anulocytes.PhysiologicalhaemolysisNormalnewbornshavehigherhemoglobin(HB)andhematocritlevelsandashortenedsurvivalperiodofthefetalRBCscontributestothedevelopmentofphysiologicanemia.Physiologicalhaemolysiserythropoiesisabruptlyceaseswithonsetofrespirationatbirth,whenthearterialoxygensaturationrisestowa
5、rd95%.levelsoferythropoietin(EPO)arelow.EPOhasadecreasedhalf-lifeandanincreasedvolumeofdistributioninnewborns.AshortenedsurvivalofthefetalRBCalsocontributestothedevelopmentofphysiologicanemia.thesizableexpansionofbloodvolumethataccompaniesrapidweightgainduringthefirst3mooflifeaddstotheneedforincreas
6、edRBCproduction.bloodcharacteristicsagesredbloodcells(RBC)andHbPhysiologicalhaemolysisandanemiawritebloodcells(WBC)andclassification4-6crossPlatelets150-250109/Lbloodvolume8-10%Redbloodcell(RBC)Termnewbornshavearedcellmassthatishigherthanatanyothertimeoflife.anappropriateconditionforthelowoxygenenvi
7、ronmentofintrauterinelife.TheRBCcountis5.010127.01012,hemoglobinconcentrationisabout150220g/Latbirth.TheRBCandhemoglobinconcentrationinpreterminfantsareslightlylowerthanthoseinterminfants.Redbloodcell(RBC)Thewiderangeofhemoglobinconcentrationisaccountedforby:Variationinhowrapidlytheumbilicalcordiscl
8、amped.Aninfantspositionafterdelivery.Ifcordclampingisdelayedandthebabyisheldlowerthanplacenta,bothhemoglobinandbloodvolumeareincreasedbyaplacentaltransfusion.ChangeofHBafterbirthReticulocyteReticulocyteReticulocyteis0.04-0.06inthefirst3days.Reticulocytedecreasesto0.005-0.015after4-7days.Reticulocyte
9、risesto0.02-0.08in4-6weeks.Reticulocyteisequaltoanadultsafter5months.Whitebloodcell(WBC)ThenormalnumberofWBCishigherininfancyandearlychildhoodthanlaterinlife.WBCcountis1510920109atbirth.After612hours,itriseto2110928109andthenbeginstodecreaseto12109by1week.WBCcountmaintainsabout10109atinfantperiodand
10、approachadultsWBCcountlevelby8years.Whitebloodcell(WBC)ThechangeinWBCclassificationistheproportionbetweenlymphocyteandgranulocyte.Lymphocyteisabout30%andgranulocyteisabout65%atbirth,butthelaterlymphocytecontrarytoneutrophilegranulocytedecreases.Theproportionbetweenlymphocyteandgranulocyteisequalat46
11、daysafterbirth.Whitebloodcell(WBC)Lymphocyteisabout60%andgranulocyteisabout35%subsequently.Theyareequalat46years.After7yearswhitecellclassificationininfantsissimilartothatinadult.4-6DaysGranulocyteLymphocyte4-6yearsChangeofproportioninLymphocyteandGranulocytePlateletcountNormalvaluefortheplateletcou
12、ntareabout150250109/Landvarylittlewithage.BloodvolumeBloodvolumeininfantsismorethaninadults.Thenewbornsbloodvolumeis10%ofhisweightandabout300mlonaverage.Achildsisabout8%10%ofhisweight.AnemiaDefination:Anemiaisdefinedasareductionoftheredbloodcellvolumeorhemoglobinconcentrationbelowtherangeofvaluesocc
13、urringinhealthypersons.Anemiaisanabsolutedecreaseinhematocrit,hemoglobinconcentration,ortheRBCcount.Anemiaisnotadiagnosis,butasignofunderlyingdisease.ThecriteriaofanemiaAgeHbconcentration28days145g/L14months90g/L46months100g/L6months6years110g/L614years120g/LAnemia1.Classification1）degree:mildmodera
14、tesevereVerysevere2）MorphologyofRBC3） Causes: lost blood , hemolytic ,deficiencyofformingHbandRBCdegreeRBC(van/mm3)Hb(g/L)Mild300-40090-110Moderate200-30060-90Severe100-20030-60Verysevere10030Morphologynanemia with microcytosis and hypochromianAnemia with macrocytosisnAnemia with normalcytosis Anemi
15、aMoreanemiaMCVMCHMCHCNormal80-9428-3232-38Micro-hypochromia8028943232-38microcytosis802832-38meancorpuscularvolume(MCV),meanscorpuscularhemoglobin(MCH),meancorpuscularhemoglobinconcentration(MCHC)Causes1.lostblood:acutechronic2.hemolysisIntrinsicmembranehereditaryspherocytosisGlycolysispyruvatekinas
16、ehemoglobinsicklecell,unstableHboxidationG6PDextrinsic:immune,infection,DICCauses3.deficiencyofformingHbandRBCdeficiencyofhematopoiesissubstancemedullaryhematopoiesisdisorder(Aplasticanemia)Theinhibitionofhaematopoiesisinducedby:InflamationChronicnephritisToxicityCancercellsinvasionbonemarrowSymptom
17、sofanemiaAsymptomatic:particularlyiftheanemiadevelopsoveralongtime.Generalmanifestation:palloroftheskinandmucousmembranes,lethargy,malnutrition,growthretardation.liver,spleenandlymphnodesexpansion.Digestionsystem:anorexia,nauseaandconstipation.SymptomsofanemiaCardiovascularandrespiratorysystem:tachy
18、cardias,increasedarterypressure,wheezeandincreasedpulse.severeanemiamaycauseheartexpansionandcongestivecardiacfailure.Nerversystem:vertigo,tinnitus,irritability,anddisordersofattention.2.DiagnosisHistorypositivemanifestationlaboratorytestsBloodsmearBMHbananysisGrowth development nutrition nails fair
19、s liverspleenandlymphnotes5 points: age, course, symptoms, feeding, pastmedicalhistory,familyhistoryMorphologyofRBC,reticulocytecount,WBC,plateletcount,bonemarrowcellsmear,HB,specialexamination3.TreatmentEliminationetiologyGeneralMedicineIntravenousbloodTransplantations:BM,stemcellsOthernutritionala
20、nemiawithmicrocytosisandhypochromiaDefinitionnutritionalirondeficiencyanemia(IDA)Hb、mostcommon、6-24ms、specialpreventionIronmetabolismIroncontentanddistribution:2/3oftheironispresentinHBand1/3intissueandtransportform.Contentofelementaliron(mg/kg)Adultfemales40Adultmales50newborn75IronmetabolismIronab
21、sorption:Theprimaryregulatorofironhomeostasisisintestinalironabsorption.Ironabsorptiontakesplaceprimarilyintheduodenumbytheenterocytesatthetipoftheintestinalvilla.Ironmustpassthoughtheapicalandthethenthebasolateralmembranesofthesecellstoreachthecirculation.IronmetabolismIronstorage:Mostbodyironiscon
22、tainedinHB,withsmalleramountsboundtoferritin(铁蛋白)andhemosiderin（含铁血黄素）inthereticuloendothelialsystem,myoglobininmuscle,circulatingtransferring,andiron-containingenzymes.Themajorironstoresareintheformofferritin.Asironcontinuestoaccumulateinthecell,asecondstorageform,hemosiderinappears.IronmetabolismI
23、roncharacteristics:Thefetusabsorbsironfromthemotheracrosstheplacenta.Terminfantshaveadequatereservesforthefirst4monthsoflife.Preterminfantshavelimitedironstoresandbecauseoftheirhigherrateofgrowth,theyoutstriptheirreservesby8weeksofage.IronmetabolismIroncharacteristics:Atbirth,becauseof“physiological
24、haemolysis”,muchironisreleasedtoplasmaandlittleironisabsorbedfromfood,Duringthesecondstage(about2monthsold),hematopoiesisisincreasedandmoreironisabsorbedfromfood,soirondeficiencyisrareinthisstage.After4months,developmentincrease,ironinfoodisdeficientandironstoresexhaust,somostirondeficiencyanemiaocc
25、ursin6monthsto2yearsor3yearsoldchild.causes1.inadequateironstores:preterminfant,twin2.intakeirondeficiency3.growthanddevelopmentincreasedironrequirement4.ironabsorbabnormal5.a amount of iron loss: hookworm infestation,repeated venesection, Meckels diverticulum,recurrentepistaxis(反复鼻出血).pathogenesisI
26、RONHbmicrocytosisandhypochromiaRBCThreestageofirondeficiencyDeficiencyofironprogressesinstagesirondepletion(ID):tissueironstoresaredeleted,undernormalcondition,thiscorrelatesdirectlywithdecreaseintheferritinlever,reticulocytepercentagedecreases.Iron deficient erythropoiesis(IDE):loss of circulatingi
27、ron.Lowserumironlessthan30ug/dl,lowtransferringsaturationand/orelevatedtotalironbindingcapacity.Threestageofirondeficiencyirondeficiencyanemia(IDA):irondeficiencyfollowing depletion of both marrow store andcirculatingiron.IDIDEIDAclinicalmanifestation1.general manifestation: mild iron deficiency isA
28、symptomatic , pallor of the skin and mucousmebranesaremostevidentandlethargy,malnutrition,growthretardation.2.liverspleenandlymphnodesenlarge3.digestionsystem:anorexia（食欲差）,nausea（恶心）,constipation（便秘）.diarrheaclinicalmanifestation4.cardiovascularandrespiratorymanifestation:tachycardia,increasedarter
29、ypressure,wheeze,increasedpulse.Severeanemiamaycauseheartexpansionandcongestivecardiacfailure.5.nervoussystemmanifestation:vertigo,irritability.clinicalmanifestationMainsignsmaybepalloroftheskinandmucousmembranes.Severeanemiamaycausecongestivecardiacfailure.IDAininfancyandearlychildhoodisassociatedw
30、ithdevelopmentaldelayandpoorgrowth.laboratorytest1.bloodsmear2.bonemarrow3.ironmetabolismInequality of size of erythrocytes，small cell，Central olistherozone obviously hypercellular,erythroidhyperplasia,thedevelopmentofcytoplasmfallsbehindnucleus.leukocytesandmegakaryocytesarenormal.Bonemarrowironsta
31、in：ferruginationgrainsintheerythocytes.Normalbonemarrowironstain正常骨髓铁染色正常骨髓铁染色IDAironstain铁缺乏骨髓铁染色铁缺乏骨髓铁染色laboratorytestThedecreaseofHBconcentrationismorethanthedecreaseofredcellscount.Bloodsmearrevealsthemorefeatureofmicrocyteandhypochromia.MCV80fl,MCH26pg,MCHC0.31.Reticulocyteisnormalorslightlydec
32、reases.WBCandplateletsarenormal.BloodcountinirondeficiencyHB75g/L120g/LRBC3.541012/L4.241012/LMCV64fl86flMCHC18.5pg32pgreticulocyte1.3%1.4%WBC7.54109/L7.64109/Lproportionnormalnormalplatelet254109/L257109/LlaboratorytestBonemarrowrevealsincreasedbasophilicnormoblastandpolychromaticnormoblast.Granulo
33、cytesystemandmegakaryocytesystemarenormal.IronmetabolismsSerumferritin(SF)(血清铁蛋白)Freeerythrocyteprotoporphyrin(FEP)Serumiron,totalironbindingcapacityIroninbonemarrowIronmetabolismsIronstudyIDIDEIDASerumferritin(SF)IronstoreRedbloodcellprotoporphyrin(FEP)NPercentsideroblastsNSerumironNN/diagnosisfirs
34、t consider - history + clinicalmanifestation+bloodsmearDecidediagnosis-bonemarrow+ironmetabolismMaybeseetreatmentwithiron(Thebonemarrowishypercellular,witherythroidhyperplasia, the normoblasts may have scanty, and thedevelopment of cytoplasm falls behind one of nucleus.leukocytesandmegakaryocytesare
35、normal.)treatment1.nursingfeeding2.getridofetiology3.ironmedicine4.interfusionsbloodOral administration of simple ferrous salts ferrous sulfate(硫酸亚铁) ferrous gluconate（葡萄糖酸亚铁）ferrous fumaratepolysaccharide iron Dosage: 4-6mg/kg elemental iron per day Oral iron preparation Administration the iron pri
36、or to meals /between to meals.Administration ascorbic acid with iron preparation. Therapeutic course: withdrawal of iron preparation 6-8 weeks after hemoglobin recover to normal level or when SF(Serum ferritin) and FEP(Free erythrocyte protoporphyrin) is normal. Oral iron preparationParenteral iron
37、preparation Tobeadministeredonlyforgastrointestinal malabsorption or severeintolerance prevents effective oral irontherapy.Parenteral iron preparationA parenteral iron preparation (iron dextran) is an effective form of iron and is usually safe when given in a properly calculated dose, but the respon
38、se to parenteral iron is no more rapid or complete than that obtained with proper oral administration of iron, unless malabsorption is a factor.BloodTransfusionWith a severe anemia, immediate red blood cell transfusion may advisable, especially in cardiac failure or severe infection, but volume and
39、speed of transfusion must be controlled well. We may transfuse, severely anemia children should be given only 2-3ml/kg of packed cells at any one time. If there is evidence of frank congestive failure, a modified exchange transfusion using fresh-packed RBCs should be considered. IrontherapyNotice :
40、3 points1.Injection iron in danger 2.Reaction : 12-24h(irritability ,appetite )- 36-48h(erythroid hyperplasia )-48-72h(reticulocytosis)-5-7ds(peaking ) 2-3ws to reticulocytes3.Times: 6-8wsPrevention4 pointsmother milk feeding specter food with iron preterm infantNutritionalmegaloblasticanemia Folic
41、acid and vitamin B12 deficiency are primary causes of megaloblastic anemia. The clinical features include anemia， the decrease of red cell is more than that of HB， the volume of red cell is larger than normal.Causes1.lessintake2.absorbabnormal3.druginteractions4.requirementincreasedPathogenesisfolic
42、acidfolicacidwith4hydratevitaminB12DNAHbverylargeRBCMegaloblasticwithLotofHbdihydrofolatereductase(THFA)VitaminB12isimportanceinsynthesisofnerve.deficiencyofvitaminB12canleadtodiscordofneurologypsychology.InthemacrocyticanemiaproducedbydeficiencyofvitaminB12,thesymptomsandsignsincludethoseofanemiaan
43、dneuropathy.nVitaminB12deficiencyneurologypsychologysymptomPatientsdevelopademyelinatinglesionofneuronsofthespinalcolumnandcerebralcortex.Thisconditionresultsinparesthesiasofthehandsandfeet,unsteadinessofgait,andeventuallymemorylossandpersonalitychanges.Thereisretardofintellectiveandphysicaldevelopm
44、ent.TremblingofExtremitiesorhead,hypertensionofmuscle,tendonreflexreinforcement,positiveBabinskissignmayappear.Clinicalmanifestation1.General features: puffiness, poor nutrition,hair yellowed, mild edema, petechia (plt),mucocutaneoushemorrhage.2.featureofanemia:lethargy,extramedullary3.neurologypsyc
45、hology:irritability,vertigo.4.digestive symptoms :anorexia, nausea,diarrhea.Laboratorytests1.bloodsmear2.bonemarrow3.bloodbiochemistrytests4.othersvariation in BRC shape and size, macrocytosis , reticulocyte count is low , nucleated RBCs and megaloblastic morphology are often seen , thrombocytopenia
46、 Hypercellular , Megaloblastic changes, hypersegmentation LaboratorytestsBloodroutineexamination:macrocyticanemia,thedecreaseofredcellcountismorethanthedecreaseofHB.MCV94fl,MCH32pg.Rreticulocyteisdecrease.WBCandplateletsarealsodecreased.Bonemarrow:increasedbasophilicnormoblastandpolychromaticnormobl
47、astic.Granulocyticsystemandmegakaryocytesystem:normal/lessthannormal.LaboratorytestsVitamineB12:normalserumvitaminB12levelsrangefrom200-800ng/L,B1212ng/LrevealsB12deficiency.Folate:normalserumfolatelevelsrangefrom5-6ug/L,folate3ug/Lrevealsdeficiency.others:LDH:serumlacticdehydrogenase(LDH)isincrease
48、Diagnosisfirst consider - history + clinicalmanifestationMarked symptoms and signs ofcentralnervoussystem.(itsupportsdefiencyofvitaminB12.)+.bloodsmeardecidediagnosis-.bonemarrow+metabolism(TodistinguishthedeficiencyoffolicacidwiththedeficiencyofvitaminB12.)maybeseetreatmentwithmedicineTreatment1.nu
49、rsingfeeding2.getridofetiology3.medicinevitB12,folicacidVitaminB12preparationVit B12 preparation to treat vit B12 deficiency. Not to use folic acid preparation in patients with vitB12 deficiency only.Intramuscular administration of vit B12 0.5-1 mg， QW or 100g，BiW，usually with reticulocytosis in 2-4
50、 days, unless there is concurrent inflammatory disease. If there is evidence of neurologic involvement, 1 mg should be injected intramuscularly daily for at least 2 wk. Maintenance therapy is necessary throughout a patients life；monthly intramuscular administration of 1 mg of vit B12 is sufficient.F
51、olicacidpreparation.Folic acid may be administered orally in a dose of 15 mg/24 hr. If the specific diagnosis is in doubt, 125 mg/24 hr of folate may be used for a week as a diagnostic test. vitamine C will help absorption of folate.IDAmegaloblasticanemiaage6m-2y6m-2yCauseironVtB12/folicacidClinical
52、manifestationpallorpuffinessextramedullary NervoussystemslightstrikingIDAmegaloblasticanemiaBloodsmearmicro-hypochromiamacrocytosisBonemarrowcytoplasmfallsbehindnucleusMegaloblasticchangesIronmetabolismabnormalNVtB12/folicacidN In this case, macrocyticanemiaiscausedbydeficiencyofvitaminB12.Why? In t
53、he macroblastic anemia produced by deficiency of vitamin B12, the symptoms and signs include those of anemia and neuropathy. Patients develop a demyelinating lesion of neurons of the spinal column and cerebral cortex. This condition results in paresthesias(感觉异常) of the hands and feet, unsteadiness of gait, and eventually memory loss and personality（智力） changes. There is retard of intellective and physical development. Trembling（震颤） of Extremities or head, hypertension of muscle, tendon reflex reinforcement, positive Babinskis sign may appear. Final diagnosisDiagnosis TreatmentTherapy
