溶血性贫血总体介绍.ppt

,第六篇血液系统疾病,第六章溶血性贫血,弋矶山医院血液内科黄东平,溶血性贫血（HA）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,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。,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,（一）红细胞自身异常所致的HA,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,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,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,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,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,（二）红细胞外部异常所致的HA,免疫性HA血管性生物因素理化因素,免疫性HA,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,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,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,（三）临床表现,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。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、黄疸和肝脾肿大三个特征胆石症、肝功能损害严重溶血时骨髓腔扩大，X线骨皮质变薄、骨骼变形,（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,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,PrimaryLaboratoryInvestigation,TheincreasedreticulocytecountisusuallyaccompaniedbyperipheralsmearRBCpolychromasia,Morphology,Pathophysiology,ClinicalRelevance,RedBloodCellMorphologyInter-relationshipTriad,MorphologyinHA,MorphologyspecifictothetypeofHAmaybepresent,e.g.,spherocytes,elliptocytesandstomatocytes,SecondaryLaboratoryInvestigation,Decisionsaboutwhatprocedurestouseforsecondary,orfollow-up,investigationarebasedonthefindingsofthehemogramandmorphology,SecondaryLaboratoryInvestigation,ChemistryBilirubin:Increasedunconjugatedbilirubinisindicativeofhemolysis,SecondaryLaboratoryInvestigation,Chemistry,continuedHaptoglobinCarriesfreeplasmaglobinDecreasedlevelsareindicativeofhemolysisMaybeincreasedininflammationmaskinghemolysis,SecondaryLaboratoryInvestigation,Chemistry,continuedIncreasedfreeplasmahemoglobinIncreasedlacticdehydrogenaseUrinalysisHemoglobinuriaormethemoglobinuriaHemosiderinuriaUrobilinogen,SurveyofHAbyType,HereditaryAcquired,HereditaryHA,MembraneabnormalitiesEnzymopathiesPlasmaconstituentabnormalities,RBCMembraneStructure,+,MembraneAbnormalities,SpherocytosisElliptocytosisPyropoikilocytosisStomatocytosis,MembraneAbnormalities,Seefigure17-1,Spleen:EffectsonRBCs,HereditarySpherocytosis,Defect:RBCproteindefectsaffectingspectrinPathophysiologyIncreasedNa+permeabilityRBCrigidityDestructioninspleenTesting:IncreasedosmoticfragilityIncubatedosmoticfragility,HereditarySpherocytosis,Seefigures17-2and3,SpherocyteMorphologyincludessectiononacutehemolyticanemia)WorkupofAnemia:,WWWSitesofInterest,Anemias:/class