《血液系统疾病》课件.ppt

血液系统疾病,Bloodsystemdisease,FeaturesOfHematopoiesisAndBloodInChildren，NutritionalAnemia,小儿造血、血像特点、营养性贫血,Abstract,FeaturesOfHematopoiesisAndBloodInChildrenAnemiaInChildrenGeneralIntroductionNutritionalirondeficiencyanemia(IDA)NutritionalMegaloblasticAnemia,purpose,TounderstandfeaturesofhematopoiesisandbloodinchildrenTocomprehendclinicalfeatures,diagnosisandtherapyofanemia.Tounderstandthedefinition,gradedivisionandclassificationofanemiainchildren.Tomasteretiology,pathogenosis,diagnosis,therapyandpreventionofnutritionalirondeficiencyanemiaandnutritionalmegaloblasticanemia.,Featuresofhematopoiesisinchildren,小儿造血特点,Featuresofhematopoiesisinchildren,一、Hematopoiesisinfetalperiod（胎儿期造血）Developmentalhematopoiesisoccursinthreeanatomicstagesmesoblastic（中胚层）,hepatic（肝）,andmyeloid（骨髓）.,Hematopoiesisinfetalperiod,yolksac,liver,spleen,Bonemarrow,lymphnode,TimeofHematopoiesis,Bloodcell,FeaturesofHematopoiesisinchildren,二、HematopoiesisafterbirthHematopoesisinbonemarrowExtramedullaryhematopoiesis（骨髓外造血）,Hematopoesisinbonemarrow,redbonemarrow（红骨髓）：ininfantandtoddlerperiodYellowbonemarrow（黄骨髓）：after57y,潜在造血功能,Hematopoesisinbonemarrow,Yellowbonemarrow（黄骨髓）cancomebacktoredbonemarrow（红骨髓）whenhematopoeticneedincreasesYellowbonemarrowisdeficiencyinchildren,especiallyininfantandtoddlerperiod,Extramedullaryhemopoiesis（髓外造血）,Whenhematopoieticdemandincreases,liver,spleenandlymphnodescomebacktothestatustoproducebloodcellshepatomegaly（肝肿大）andsplenomegaly（脾肿大）appearsandmaybethereareimmatureerythrocytesandgranulocytesincirculatingbloodExtramedullaryhemopoiesisisthespecificphenomenaonlyappearingininfantandtoddler,Extramedullaryhemopoiesis（髓外造血）,年龄：ininfantandtoddler病因：Whenhematopoieticdemandincreases.E.g.anemia（贫血）造血部位：liver,spleenandlymphnodescomebacktothestatustoproducebloodcells体征：hepatomegaly（肝肿大）andsplenomegaly（脾肿大）,Extramedullaryhemopoiesis（髓外造血）,血象：thereareimmatureerythrocytesandgranulocytesincirculatingblood,Featuresofbloodinchildren,小儿血象特点,血容量bloodcapacitance,新生儿：占体重10%儿童：占体重8-10%成人：占体重6-8%,Erythrocyteandhemoglobin,Erythrocyteandhemoglobin,Hemoglobin,Atbirth,1yr,2yr,Hemoglobin,Leucocytecount,0,0,0,0,0,Leucocytecount,DifferentialcountofWBC(白细胞分类计数),DifferentialcountofWBC(白细胞分类计数),血小板bloodplatelet,出生时偏低，以后增多（但10万）正常：10-30万,AnemiaInChildren-GeneralIntroduction,小儿贫血总论,贫血的定义Definitionofanemia,外周血中单位容积内的红细胞数、血红蛋白量或红细胞压积低于正常值但临床上通常用血红蛋白量低于正常值来诊断贫血,Thecriterionofanemia,海拔每升高1000米，血红蛋白上升4%,生理性贫血PhysiologicAnemia,定义特指生后23月且无病理性因素存在时,RBC3.0 x1012/L、Hb铁摄入吸收障碍：搭配不合理/慢性腹泻/反复感染丢失过多：长期慢性失血，0.5mg/ml(牛奶过敏、息肉、钩虫、憩室),发病机制,缺铁对血液系统影响,铁,原卟啉,血红素,珠蛋白,Hb,细胞分裂增殖,RBC数量,小细胞低色素贫血,发病机制,缺铁对非造血系统影响肌红蛋白合成含铁酶活性,体力神经系统消化道免疫力,铁缺乏演变过程,红细胞生成缺铁期,缺铁性贫血期,铁减少期,储存铁:SF骨髓外铁肠道铁吸收储存铁:SFSITSTIBCFEPHb正常临床贫血,亚临床型,ID,IDE,IDA,临床表现,婴幼儿，起病缓慢血液系统症状贫血一般表现：苍白、疲乏、头晕髓外造血：肝脾淋巴结肿大,3.非造血系统症状,消化系统,神经系统,心血管系统,免疫运动系统,实验室检查,外周血象小细胞低色素RBC大小不等，以小为主，中央淡染区扩大MCV、MCH、MCHCWBC、BPC多正常,SmearofBlood（血涂片）,A.正常外周血象,B.ID期,C.IDE期,D.轻度IDA期,E.中度IDA期,F.重度IDA期,实验室检查,骨髓象增生活跃，胞浆发育落后于胞核粒系、巨核系正常胞外铁减少、胞内铁粒细胞数15%,Bonemarrow,A.有核细胞增生明显活跃,B.浆幼核老,实验室检查,3.生化检查血清铁蛋白(SF)500ug/dl血清铁(SI)350ug/dl,诊断,喂养史临床表现外周血象,铁代谢检查,铁剂治疗有效,骨髓Hb电泳,初步诊断,确定诊断,证实诊断,鉴别诊断,治疗,原则祛除病因补充铁剂铁剂：硫酸亚铁、富马酸铁、葡萄糖酸亚铁剂量：铁元素46mg/kg.d两餐之间促进吸收：VitC疗程：Hb正常后68w,治疗,治疗反应：1224h3d1w12w34w继用68w2.无效原因：用药？病因？误诊？,Hb正常,Hb,Ret,精神症状,增加铁储存,间隔补铁WHO,定义是指q3d或qw补铁一次理论基础小肠粘膜细胞更新周期为56d动物实验铁生物利用率q3d=2.6倍qd临床研究Hb升高与胃肠道副反应推荐小剂量(元素铁2mg/kg.d)短疗程(12w)间隔补铁(qw),Parenteraliron,Tobeadministeredonlyforgastrointestinalmalabsorptionorsevereintolerance（严重不耐受）preventseffectiveoralirontherapy.,Reticulocytosis（网织红细胞）,一般治疗34天后，网织红细胞开始升高。,输血Transfusion,严重贫血伴有心功能不全者，可考虑输浓缩红细胞，但应少量及慢速。,预防,提倡母乳喂养及时添加辅食早产儿2月后补铁治疗慢性病,Prevention,NutritionalMegaloblasticAnemia（营养性巨幼红细胞性贫血）,FolicacidandvitaminB12deficiencyareprimarycausesofmegaloblasticanemia.,Case2.,Zhou,male,8gressivedrowsinessandgoasleepfor20hrpreday.Hecannotsmileandtoraisehishead,whichcanbedonebefore.HewasfedwithMothersmilkonly,3timeseveryday.,Case2.,Physicalexamination：emotionalexpressionSluggishness（呆滞）,Pallor.nojaundice,rashesandhemorrhagicspotsinskin;pallorinhislips.Breathsoundsaredistinctandtherearenoraleinlungs.Heartrateis110timesperminute.Theliverandspleenenlargeto3centimetersand4centimetersbelowlowercostamarginrespectively.,Bloodroutine,questions:,1.Whichkindofanemiaitbelongs?,Answer,Theclinicdatashowsamacrocyticanemia.,questions:,1.Whichkindofanemiaitbelongs?,questions:,2.IsitcausedbydeficiencyoffolicacidorvitaminB12?,Answer,Inthiscase,macrocyticanemiaiscausedbydeficiencyofvitaminB12.,Why?,questions:,3.whatisthedifferencesinclinicalmanifestationbetweendeficiencyoffolicacidanddeficiencyofvitaminB12?,定义,由于VtB12/叶酸缺乏所致的大细胞性贫血，其临床特点是贫血、神经精神症状、RBC胞体变大、骨髓出现巨幼细胞、VtB12/叶酸治疗有效,病因,摄入不足：母乳(母挑食)、羊乳(叶酸)、辅食吸收障碍:内因子B12、小肠病变、药物需要增加：生长发育快、慢性溶血消耗过多或代谢障碍：感染、酶缺乏,营养性巨幼细胞性贫血发病机理,Answer,InthemacroblasticanemiaproducedbydeficiencyofvitaminB12,thesymptomsandsignsincludethoseofanemiaandneuropathy.Patientsdevelopademyelinatinglesion（脱髓鞘损害）ofneuronsofthespinalcolumnandcerebralcortex（大脑皮层）.Thisconditionresultsinparesthesias(感觉异常)ofthehandsandfeet,unsteadinessofgait（步态不稳）,andeventuallymemorylossandpersonality（智力）changes.Thereisretardofintellectiveandphysicaldevelopment（智力及体格发育延迟）.Trembling（震颤）ofExtremities（四肢）orhead,hypertensionofmuscle,tendonreflexreinforcement（腱反射亢进）,positiveBabinskissignmayappear.,临床表现,婴幼儿多见，起病缓慢贫血：一般表现，髓外造血营养不良：皮肤苍黄、虚胖神经系统：反应迟钝、智力落后/倒退、震颤,实验室检查,1.外周血象大细胞正色素贫血RBC：大小不等、以大为主，嗜多色/嗜点彩中性粒细胞：变大、过分叶(早期诊断)Ret、BPC减少,Smearofblood（血涂片）,Smearofpatientblood,A.巨幼细胞贫血血象,B.嗜碱性点彩红细胞,实验室检查,2.骨髓象增生明显活跃，红系为主粒红系巨幼变，巨核系过分叶3.血清B12/叶酸浓度下降,BM(骨髓),A.有核细胞增生明显活