溶血性贫血的介绍.ppt
皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室第六篇第六篇 血液系统疾病血液系统疾病皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室第六章第六章 溶血性贫血溶血性贫血弋矶山医院血液内科黄东平皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室溶血性贫血（溶血性贫血（HAHA）概述）概述定义临床分类临床表现发病机制与实验室检查诊断和鉴别诊断治疗皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室一、定义一、定义溶血是一组由于后天或先天的各种原因使红细胞遭破坏寿命缩短的过程。HA系指红细胞破坏超过骨髓造血代偿功能而发生的一种贫血。如溶血发生而骨髓能代偿时，临床无贫血，称为溶血性疾病溶血性疾病。皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室二、临床分类二、临床分类红细胞自身异常所致的HA红细胞外部异常所致的HA 皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室（一）红细胞自身异常所致的（一）红细胞自身异常所致的HAHA红细胞膜异常遗传性红细胞酶的缺乏遗传性珠蛋白生成障碍血红素异常 皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室红细胞膜异常红细胞膜异常遗传性红细胞膜缺陷 遗传性球形红细胞增多症、遗传性椭圆形红细胞增多症、遗传性棘性细胞增多症、遗传性口形细胞增多症获得性血细胞膜糖化肌醇磷酯（GPI）锚链膜蛋白异常，如PNH 皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室遗传性红细胞酶的缺乏遗传性红细胞酶的缺乏戊糖磷酸途径酶缺陷 如G6PD缺乏症等无氧糖酵解途径缺陷 如丙酮酸激酶缺乏症等核苷代谢酶系、氧化还原酶系等缺陷等皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室遗传性珠蛋白生成障碍遗传性珠蛋白生成障碍珠蛋白肽链结构异常不稳定血红蛋白病、血红蛋白病S、D、E等珠蛋白肽链数量异常地中海贫血皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室血红素异常血红素异常先天性红细胞卟啉代谢异常如红细胞生成性血卟啉病，原卟啉型、尿卟啉型和粪卟啉型铅中毒可影响血红素的合成发生HA皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室（二）红细胞外部异常所致的（二）红细胞外部异常所致的HA HA 免疫性HA血管性生物因素理化因素皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室免疫性免疫性HAHA自身免疫性HA温抗体型或冷抗体型（冷凝集型、D-L抗体型）；原发性或继发性（SLE、病毒或药物等0同种免疫性HA如血型不合的输血反应、新生儿HA皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室血管性血管性微血管病性HA如（TTP/HUS）、DIC、败血症等瓣膜病：钙化性主动脉瓣狭窄、人工心瓣膜和血管炎等血管壁受到反复挤压：行军性血红蛋白尿皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室生物因素、理化因素生物因素、理化因素蛇毒、疟疾和黑热病等大面积烧伤、血浆中渗透压改变和化学因素如苯肼、亚硝酸盐等中毒，可引起获得性高铁血红蛋白血症而溶血皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室（三）临床表现（三）临床表现急性溶血：起病急，突然寒战、高热、头痛、急性溶血：起病急，突然寒战、高热、头痛、四肢酸痛等，面色苍白、血红蛋白尿和黄疸。四肢酸痛等，面色苍白、血红蛋白尿和黄疸。严重者周围循环衰竭和急性肾衰竭严重者周围循环衰竭和急性肾衰竭慢性溶血：起病慢，常有贫血、黄疸和肝脾肿慢性溶血：起病慢，常有贫血、黄疸和肝脾肿大三个特征大三个特征胆石症、肝功能损害胆石症、肝功能损害严重溶血时骨髓腔扩大，严重溶血时骨髓腔扩大，X X线骨皮质变薄、骨线骨皮质变薄、骨骼变形骼变形皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室（四）发病机制和临床表现（四）发病机制和临床表现红细胞破坏和血红蛋白降解红系代偿性增生红细胞具有缺陷或寿命缩短皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室红细胞破坏和血红蛋白降解红细胞破坏和血红蛋白降解血管内溶血游离Hb升高（40mg/L）结合珠蛋白降低（3皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Primary Laboratory InvestigationThe increased reticulocyte count is usually accompanied by peripheral smear RBC polychromasia皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室MorphologyPathophysiologyClinical RelevanceRed Blood Cell MorphologyInter-relationship Triad皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Morphology in HAMorphology specific to the type of HA may be present,e.g.,spherocytes,elliptocytes and stomatocytes皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Secondary Laboratory InvestigationDecisions about what procedures to use for secondary,or follow-up,investigation are based on the findings of the hemogram and morphology皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Secondary Laboratory InvestigationChemistryBilirubin:Increased unconjugated Bilirubin:Increased unconjugated bilirubin is indicative of hemolysisbilirubin is indicative of hemolysis皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Secondary Laboratory InvestigationChemistry,continuedHaptoglobinHaptoglobin Carries free plasma globinCarries free plasma globin Decreased levels are indicative of Decreased levels are indicative of hemolysishemolysis May be increased in inflammation masking May be increased in inflammation masking hemolysishemolysis皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Secondary Laboratory InvestigationChemistry,continuedChemistry,continued Increased free plasma hemoglobinIncreased free plasma hemoglobin Increased lactic dehydrogenaseIncreased lactic dehydrogenase UrinalysisUrinalysis Hemoglobinuria or methemoglobinuriaHemoglobinuria or methemoglobinuria HemosiderinuriaHemosiderinuria UrobilinogenUrobilinogen皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Survey of HA by TypeHereditaryAcquired皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Hereditary HAMembrane abnormalitiesEnzymopathiesPlasma constituent abnormalities皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室RBC Membrane StructureRBC Membrane Structure+PlasmaIntegralProteinsPeripheral ProteinsLipid Bi-Layer Lipid Bi-LayerRBC Cytoplasm皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Membrane AbnormalitiesSpherocytosisElliptocytosisPyropoikilocytosisStomatocytosis皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Membrane AbnormalitiesSee figure 17-1皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Excess membrane cholesterolRBC inclusionsSenescent RBCComplement coated RBCAntibody coated RBCRigid RBCSpleen:Effects on RBCsSpleen:Effects on RBCs皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Hereditary SpherocytosisDefect:RBC protein defects affecting spectrinPathophysiologyIncreased Na+permeability Increased Na+permeability RBC rigidity RBC rigidity Destruction in spleenDestruction in spleenTesting:Increased osmotic fragilityIncubated osmotic fragility 皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Hereditary SpherocytosisSee figures 17-2 and 3皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Spherocyte Morphology&Spherocyte Morphology&PathophysiologyPathophysiologyLacks area of central pallorLacks area of central pallorLacks area of central pallorLacks area of central pallorAdditional causesAdditional causesAdditional causesAdditional causes Immunologic reactionImmunologic reactionImmunologic reactionImmunologic reaction Physical traumaPhysical traumaPhysical traumaPhysical traumaUsually 6.5 Usually 6.5 Usually 6.5 Usually 6.5 m diameterm diameterm diameterm diameterMicrospherocytes:4 Microspherocytes:4 Microspherocytes:4 Microspherocytes:25%of all RBCs)(25%of all RBCs)Macro-ovalocytes seen in Macro-ovalocytes seen in megaloblastic anemiasmegaloblastic anemiasNon-specific finding in other types Non-specific finding in other types of anemia(25%of all RBCs)of anemia(30 30 30 30 short,blunt projections evenly short,blunt projections evenly short,blunt projections evenly short,blunt projections evenly distributed over the cell surfacedistributed over the cell surfacedistributed over the cell surfacedistributed over the cell surfaceA.K.A.burr cell,crenated A.K.A.burr cell,crenated A.K.A.burr cell,crenated A.K.A.burr cell,crenated RBC&sea urchin cellRBC&sea urchin cellRBC&sea urchin cellRBC&sea urchin cellWet mount comparisonWet mount comparisonWet mount comparisonWet mount comparison皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Acanthocyte MorphologyAcanthocyte Morphology5-12 5-12 5-12 5-12 thornlike spicules unevenly thornlike spicules unevenly thornlike spicules unevenly thornlike spicules unevenly distributed over the cell surfacedistributed over the cell surfacedistributed over the cell surfacedistributed over the cell surfaceProjections may be club shaped or drumstick Projections may be club shaped or drumstick Projections may be club shaped or drumstick Projections may be club shaped or drumstick shaped(spur cells)shaped(spur cells)shaped(spur cells)shaped(spur cells)皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Acquired Hemolytic AnemiaExtracorpuscular defectsAbnormal blood vessel structureAbnormal blood vessel structureMechanical injuryMechanical injuryMiscellaneousMiscellaneousIntracorpuscular defectsImmune processes皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Extracorpuscular DefectsMicroangiopathic hemolytic anemiaDisseminated intravascular coagulation Disseminated intravascular coagulation(DIC)(DIC)Hemolytic uremic syndrome(HUS)Hemolytic uremic syndrome(HUS)Thrombotic thrombocytopenic purpura Thrombotic thrombocytopenic purpura(TTP)(TTP)皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Direction ofblood flowFibrinSchistocyteSchistocyteMicroangiopathic TraumaMicroangiopathic Trauma皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室SchistocyteSchistocyteMicroangiopathic TraumaMicroangiopathic TraumaSlides not shown.See Figure 8-12,p.94,Clinical HematologyClinical Hematology皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Intracorpuscular-PNHMajor defect-Complement sensitive RBCs in acidified plasma environmentPathophysiology-Extravascular lysis of affected RBC occurring during the evening hours leading to a chronic hemolytic anemia皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Intracorpuscular-PNHSecondary investigationScreening test-Sugar water test Screening test-Sugar water test Increased hemolysis in PNHIncreased hemolysis in PNHDefinitive test-Hams acidified serum Definitive test-Hams acidified serum test(See Table 18-1,Clinical test(See Table 18-1,Clinical Hematology,p.269.)Significant lysis in Hematology,p.269.)Significant lysis in tubes containing acidified serum and tubes containing acidified serum and complementcomplement皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Module Objectives:Blood Loss AnemiaAt the end of this module you should be able toDescribe the natural course of acute blood loss anemiaDescribe the changes in laboratory test results that can be expected in acute blood loss anemia皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Module Objectives:Blood Loss AnemiaExplain the test results expected in chronic blood loss anemia皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory FindingsBlood Loss AnemiaAcute blood loss anemia No change in CBC parameters in first No change in CBC parameters in first few hoursfew hours3-4 hours3-4 hours Hb/Hct Possible Hb/Hct Possible WBC count Possible WBC count Possible 皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory FindingsBlood Loss AnemiaAcute blood loss anemia,continued12-24 hours12-24 hours Hb/Hct Hb/Hct reflecting degree of blood loss reflecting degree of blood loss Probable leukocytosisProbable leukocytosis Possible thrombocytosisPossible thrombocytosis皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory FindingsBlood Loss AnemiaAcute blood loss anemia,continued3-5 days3-5 days Possible Possible MCV MCV Possible Possible reticulocyte count reticulocyte count Possible Possible RPI(3)RPI(3)皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory FindingsBlood Loss AnemiaSee Table 20-2皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory FindingsBlood Loss AnemiaFindings and course in chronic blood loss anemia are similar to iron deficiency anemia皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory FindingsBlood Loss AnemiaMorphology-Acute blood lossFirst few hours no significant changesFirst few hours no significant changes3-4 hours3-4 hours Possible left shiftPossible left shift Possible NRBCsPossible NRBCs皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory FindingsBlood Loss AnemiaMorphology-Acute blood loss,continued12-24 hours12-24 hours Possible left shiftPossible left shift Possible NRBCsPossible NRBCs Platelet estimate Platelet estimate 皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory FindingsBlood Loss AnemiaMorphology-Acute blood loss,continued3-5 days3-5 days Possible macrocytosisPossible macrocytosis Possible polychromasiaPossible polychromasia皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory FindingsBlood Loss AnemiaFindings and course in chronic blood loss anemia are similar to iron deficiency anemia皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室EtiologyAcute Blood Loss AnemiaTraumatic hemorrhageGI hemorrhageOperative hemorrhageRuptured aneurysmsObstetric complicationsMassive hemoptysis皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Etiology-Chronic Blood Loss AnemiaExcessive mensesGI hemorrhageColon carcinomaHookworm皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Pathophysiology-Chronic Blood Loss AnemiaPathophysiology similar to iron deficiency anemiaAnemia results from ineffective recycling of iron皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Laboratory Investigation in Chronic Blood Loss AnemiaSerum ferritin levels to assess storage iron statusOther iron studiesStool occult blood to R/O GI bleeding皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室Session Summary皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室WWW Sites of InterestAcute Blood Loss AnemiaAcute Blood Loss Anemia:http:/www.vet.purdue.edu/vpb/clinpath/vhttp:/www.vet.purdue.edu/vpb/clinpath/vpb555/555rbc3/(PowerPoint pb555/555rbc3/(PowerPoint presentations;includes section on acute presentations;includes section on acute hemolytic anemia)hemolytic anemia)Workup of AnemiaWorkup of Anemia:http:/ http:/ of test correlation for anemia;(Summary of test correlation for anemia;from Internal Medicine Board Review from Internal Medicine Board Review Course)Course)皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室皖南医学院内科学教研室WWW Sites of InterestAnemiasAnemias:http:/www1.omi.tulane.edu/classware/pathttp:/www1.omi.tulane.edu/classware/pathology/Krause/Anemias/Anemias.html hology/Krause/Anemias/Anemias.html(Overview of anemias from Dr.John(Overview of anemias from Dr.John Krauses Hematopathology Homepage,Krauses Hematopathology Homepage,Tulane University-link provided)Tulane University-link provided)
