2011血液系统疾病

血液系统疾病,Blood system disease,Features Of Hematopoiesis And Blood In Children，Nutritional Anemia,小儿造血、血像特点、营养性贫血,Abstract,Features Of Hematopoiesis And Blood In Children Anemia In ChildrenGeneral IntroductionNutritional iron deficiency anemia(IDA)Nutritional Megaloblastic Anemia,purpose,To understand features of hematopoiesis and blood in children To comprehend clinical features, diagnosis and therapy of anemia. To understand the definition, grade division and classification of anemia in children. To master etiology, pathogenosis, diagnosis, therapy and prevention of nutritional iron deficiency anemia and nutritional megaloblastic anemia.,Features of hematopoiesis in children,小儿造血特点,Features of hematopoiesis in children,一、Hematopoiesis in fetal period（胎儿期造血） Developmental hematopoiesis occurs in three anatomic stagesmesoblastic（中胚层）, hepatic（肝）, and myeloid（骨髓）.,Hematopoiesis in fetal period,yolk sac,liver,spleen,Bone marrow,lymph node,Time of Hematopoiesis,Blood cell,Features of Hematopoiesis in children,二、Hematopoiesis after birth Hematopoesis in bone marrow Extramedullary hematopoiesis （骨髓外造血）,Hematopoesis in bone marrow,red bone marrow（红骨髓）： in infant and toddler periodYellow bone marrow（黄骨髓）：after 57y, 潜在造血功能,Hematopoesis in bone marrow,Yellow bone marrow（黄骨髓） can come back to red bone marrow（红骨髓）when hematopoetic need increases Yellow bone marrow is deficiency in children, especially in infant and toddler period,Extramedullary hemopoiesis（髓外造血）,When hematopoietic demand increases, liver, spleen and lymph nodes come back to the status to produce blood cellshepatomegaly（肝肿大） and splenomegaly（脾肿大） appears and maybe there are immature erythrocytes and granulocytes in circulating bloodExtramedullary hemopoiesis is the specific phenomena only appearing in infant and toddler,Extramedullary hemopoiesis（髓外造血）,年龄：in infant and toddler病因： When hematopoietic demand increases. E.g. anemia（贫血）造血部位： liver, spleen and lymph nodes come back to the status to produce blood cells体征： hepatomegaly（肝肿大） and splenomegaly（脾肿大）,Extramedullary hemopoiesis（髓外造血）,血象： there are immature erythrocytes and granulocytes in circulating blood,Features of blood in children,小儿血象特点,血容量blood capacitance,新生儿：占体重10%儿童：占体重8-10%成人：占体重6-8%,Erythrocyte and hemoglobin,Erythrocyte and hemoglobin,Hemoglobin,At birth,1 yr,2 yr,Hemoglobin,Leucocyte count,0,0,0,0,0,Leucocyte count,Differential count of WBC (白细胞分类计数),Differential count of WBC (白细胞分类计数),血小板blood platelet,出生时偏低，以后增多（但10万）正常：10-30万,Anemia In Children-General Introduction,小儿贫血总论,贫血的定义Definition of anemia,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,The criterion of anemia,海拔每升高1000米，血红蛋白上升4%,生理性贫血Physiologic Anemia,定义 特指生后23月且无病理性因素存在时, RBC 3.0 x 1012/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 : SF SI TS TIBC FEP Hb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,Smear of Blood（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%,Bone marrow,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) 60ug/dl转铁蛋白饱和度(TS) 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： 1224h 3d1w 12w 34w 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),Parenteral iron,To be administered only for gastrointestinal malabsorption or severe intolerance（严重不耐受） prevents effective oral iron therapy.,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,Nutritional Megaloblastic Anemia（营养性巨幼红细胞性贫血）,Folic acid and vitamin B12 deficiency are primary causes of megaloblastic anemia.,Case 2.,Zhou, male, 8 month old.pallor for 2 mo . progressive drowsiness and go asleep for 20 hr pre day. He can not smile and to raise his head, which can be done before. He was fed with Mothers milk only,3 times every day.,Case 2.,Physical examination： emotional expression Sluggishness（呆滞）, Pallor. no jaundice, rashes and hemorrhagic spots in skin; pallor in his lips. Breath sounds are distinct and there are no rale in lungs. Heart rate is 110 times per minute. The liver and spleen enlarge to 3 centimeters and 4 centimeters below lower costa margin respectively.,Blood routine,questions:,1.Which kind of anemia it belongs?,Answer,The clinic data shows a macrocytic anemia.,questions:,1.Which kind of anemia it belongs?,questions:,2. Is it caused by deficiency of folic acid or vitamin B12 ?,Answer,In this case, macrocytic anemia is caused by deficiency of vitamin B12.,Why?,questions:,3.what is the differences in clinical manifestation between deficiency of folic acid and deficiency of vitamin B12?,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,In the macroblastic anemia produced by deficiency of vitamin B12, the symptoms and signs include those of anemia and neuropathy. Patients develop a demyelinating lesion（脱髓鞘损害） of neurons of the spinal column and cerebral cortex（大脑皮层）. This condition results in paresthesias(感觉异常) of the hands and feet, unsteadiness of gait（步态不稳）, and eventually memory loss and personality（智力） changes. There is retard of intellective and physical development（智力及体格发育延迟）. Trembling（震颤） of Extremities（四肢） or head, hypertension of muscle, tendon reflex reinforcement（腱反射亢进）, positive Babinskis sign may appear.,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,Smear of blood（血涂片）,Smear of patientblood,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