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1、溶血性贫血正常红细胞结构组成：7.5um生理特点 红细胞膜的通透性 红细胞的可变形性 渗透脆性 悬浮稳定性代谢 糖代谢:酵解酶、磷酸戊糖旁路功能 氧运输正常红细胞破坏120天，0.8%衰老RBC 结构异常僵硬易被吞噬 酶能力僵硬易被吞噬清除单位 脾脏（识别衰老和缺陷RBC）肝骨髓/其他红细胞成分无线粒体和细胞核 细胞膜 血红蛋白 珠蛋白 血红素 原卟啉IX 亚铁原子什么是溶血性贫血?溶血 体内红细胞过早破坏贫血 红细胞破坏超过骨髓生成 骨髓:儿童 2-3倍 成人 6-8倍 儿童更容易发生资料溶血时身体发生了什么改变?Extravascular(liver and spleen)Intravas
2、cular血管内Red cellMacrophage巨噬细胞HbglobinFeProtoporphyrinAmino acid poolUnconjugated bilirubin 未结合胆红素未结合胆红素TransferrinPlasma Hb methemoglobinGlobinMethemeHb dimerHepatocytesHemeHemopexin血结素血结素FeBilirubinConjugationFecalUrobilinogenUrine Urobilinogen尿胆元尿胆元Urine Hb血红蛋白尿血红蛋白尿Urine Hemosiderin含铁血黄素含铁血黄素Hap
3、toglobin结合珠蛋白结合珠蛋白Hb-haptoglobin complexintestineskidney粪胆元粪胆元实验室指标CBC Anemia Increased reticulocyte 网织红细胞 countHemolytic markers Increased bilirubin Increased LDH/GOT haptoglobin?Presence of hemalbumin游离血红蛋白?Hemosidenuria含铁血黄素尿?Hemoglobinuria血红蛋白尿?最常见症状苍白/皮肤颜色黄疸,皮肤、眼、口腔尿色加深发热虚弱头晕昏睡体力不耐受体征皮肤或甲床苍白脉速肝
4、或脾肿大诊断三步曲第一步 确定溶血存在1.红细胞破坏 血:贫血或球形红细胞/红细胞碎片 尿 便 生化 未结合胆红素/乳酸脱氢酶/GOT 红细胞寿命缩短第一步 确定溶血存在2.骨髓代偿增生 血片中红细胞的嗜多色性 网织红细胞增多 骨髓红细胞增多 慢性长期溶血：骨骼改变：髓腔增大特殊面容：蒙古面容血涂片ABCDEFGHIJA-NormalB-Micro/hypoC-MacroD-TargetE-SpheroF-Heinz bodyG-SchistocyteH-nRBCI-PolychromJ-Teardrop鉴别诊断黄疸 肝大压痛、消化道症状；直胆/间胆、ALT，AKP（+-）胆道梗阻 胆囊肿大，
5、大便白:直胆，ALT（+-），AKP 先天性黄疸 遗传性葡萄糖醛酸转换酶缺乏，间胆直胆,缺乏溶血+贫血依据 贫血出血 营养性 生成不良 第二步 确定溶血部位Extravascular HemolysisIngested by RE cell(spleen&liver)HemeGlobinIronProtoporphyrinReutilizedbilirubinReutilizedIntravascular HemolysisHgb liberated in blood vesselHgb+haptoglobinHgb+albuminHgb excreted in urine Serum hap
6、toglobin+hemalbumin&plasma Hgb+hemoglobinuria&hemosidenuria鉴别诊断血管外血管外血管内血管内脾脏 肿大 无肿大LDH bilirubin haptoglobin N to absent absenthemoglobinuria absent presentfree Hb in plasma absent presenturine hemosiderin absent present第三步 确定病因-1外源性 destroyed by becoming trapped in the spleen infection,or destroye
7、d from drugs 内源性 a defect within red blood cells themselves often inherited:sickle cell anemia 镰贫 and G6PD缺乏第三步 确定病因-2血管内 外源性 机械:prosthetic heart valve/march hemoglobinuria 微血管病变:DIC,TTP,HUS 免疫性 acute hemolytic transfusion reaction,PNH 感染:malaria/Clostridium welchii sepsis 内源性 酶病：G6PD缺乏第三步 确定病因-2血管外
8、 内源性 膜病 球形红细胞增多,椭圆形红细胞增多 血红蛋白病 -Sickle cell,HbC etc.Thalassemia Syndromes地中海贫血-,酶病 外源型 Immune and non-immune hemolytic anemia直接（间接）抗人球蛋白试验Patients RBCABO Rh compatible plasma+incubateIgGC3C3IgGC3IgGRabbit or human anti-IgG and/or anti-C3 AntibodyAgglutination聚集聚集=positivepatients plasmaDirect+ABO Rh
9、 compatible RBCIndirect 渗透脆性试验 EOF原理原理:测定红细胞对不同浓度低滲氯化钠溶血的抵抗力测定红细胞对不同浓度低滲氯化钠溶血的抵抗力-容积储备容积储备 -渗透脆性渗透脆性-容积储备容积储备-膜膜/容积容积 ：膜异常：膜异常-渗透脆性渗透脆性-容积储备容积储备 -膜膜/容积容积：内容物减少：内容物减少 第三步 确定病因-3思路1、寻找感染、机械损伤、微血管病变证据2、抗人球蛋白试验Coomb 实验+：免疫性溶血3、Coomb 实验 先天性溶血4、红细胞脆性试验红细胞膜疾病：遗传性球型红细胞增多症正常红细胞酶疾病血红蛋白病：地中海贫血遗传性球形红细胞增多症病因最常见的
10、红细胞膜病 1/5000 in northern European populations遗传方式 常染色体显性遗传 常染色体隐性遗传 25%no previous family history-new mutations+recessive inheritanceencode RBC membrane cytoskeleton proteins genes mutationsRED CELL CYTOSKELETONMembrane defectsModel of RBC membrane资料loss of membrane=loss of SA=loss of deformability=
11、increased splenic clearanceNormalHereditary spherocytosismembranecytoskeleton病因结构缺陷 血影蛋白/锚蛋白/band 3Deficiency surface area deficiency leading to spherocytosis lipid bilayer skeleton uncoupling membrane loss in the form of microvesicles脾窦破坏脾切除临床表现新生儿 the cause and require phototherapy or exchange tra
12、nsfusions在儿童严重程度不一 无症状 三联症：贫血+黄疸+脾大 胆色素结石:可早在4-5岁发生危象 溶血危象 再障危象：微小病毒 巨幼贫危象实验室Evidence for hemolysis Reticulocytosis 6-20%(10%)+anemia 6-10g/L-indirect bilirubin MCV n/+MCHC (36-36g/ml RBCs)reticulocytes and sperocytes:Spherocytes:microcytic小细胞+hyperchromic高色素+less central pallor 15-20%BM:erythroid h
13、yperplasiagallstone资料诊断Blood film Spherocytes and reticulocytes Spherocytes can confirmed:渗透脆性试验家族史脾肿大osmotic fragility testnegative DATRBC membrane protein analysis(80%)鉴别：自免溶贫治疗脾切除 Not recommend hb10g/dl+Ret 5-6yr Laporoscopeic splenectomy Before:vaccines for encapsulated organisms After:oral prol
14、phylactic penicillin Postsolenectomy thrombocytosisFolic acid 1mg/24hrTransfusion G6PD 酶缺乏Glycolytic PathwayGlucoseGlucose-6-phosphateATPADPFructose-6-phosphatehexokinaseGlucosephosphate isomerasecFructose-1,6-bisphosphateATPADPGlyceraldehyde-3-phosphateDihydroxy-acetone Phosphatephosphofructokinase
15、aldolaseTriosephophate isomerase.Anaerobic GlycolysisHexose Monophosphate ShuntGlucose-6-dehydrogenase6-phosphogluconate dehydrogenseNADPNADPHGlutathione reductaseReduced glutathione(anti-oxidant)Oxodozed GlutathioneGlutathione peroxidaseH2O2H2OGlutathione synthetaseGlycinecysteine Glutamic AcidGlut
16、amylcysteine synthetaseRole of G6PDGlucoseGlucose-6-phosphate6-PhosphogluconateRibose-5-phosphateFructose-6-phosphateGlyceraldehyde-3-phosphate+Glyceraldehyde-3-phosphatePentose ShuntATPADP G6PDehydrogenaseNADPHNADP+GSSGGSHGSH reductaseNADPHNADP+H2OH2O2O2CatalaseMaintaining adequate levels of NADPHNADPH back to NADP+reduction of oxidized glutathione(GSSG)to glutathione(GSH).ndetoxify the harmful oxidant H2O2.nprevent oxidative damage to hemoglobinNADPH 还原型辅酶还原型辅酶GSH 还原型谷光甘肽还原型谷光甘肽G6PD酶缺乏是发生了什么?G
