1、第六篇第六篇 血液系统疾病血液系统疾病第六章第六章 溶血性贫血溶血性贫血溶血性贫血（溶血性贫血（HAHA）概述）概述一、定义一、定义二、临床分类二、临床分类（一）红细胞自身异常所致的（一）红细胞自身异常所致的HAHA红细胞膜异常红细胞膜异常遗传性红细胞酶的缺乏遗传性红细胞酶的缺乏遗传性珠蛋白生成障碍遗传性珠蛋白生成障碍血红素异常血红素异常（二）红细胞外部异常所致的（二）红细胞外部异常所致的HA HA 免疫性免疫性HAHA血管性血管性生物因素、理化因素生物因素、理化因素（三）临床表现（三）临床表现（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细胞破坏和血红蛋白降解红细
2、胞破坏和血红蛋白降解红细胞破坏和血红蛋白降解血管外溶血的一种特殊形式血管外溶血的一种特殊形式红系代偿性增生红系代偿性增生红细胞具有缺陷或寿命缩短红细胞具有缺陷或寿命缩短鉴别诊断鉴别诊断自身免疫性溶血性贫血自身免疫性溶血性贫血温抗体型温抗体型AIHAAIHA一、病因一、病因二、临床表现二、临床表现三、实验室检查三、实验室检查三、实验室检查三、实验室检查四、诊断四、诊断实验室检查实验室检查五、治疗五、治疗冷抗体型冷抗体型AIHAAIHAANEMIAS OF EFFECTIVE ERYTHROPOIESISAnemias of Effective Erythropoiesis:Module Obje
3、ctivesAnemias of Effective Erythropoiesis:Module ObjectivesAnemias of Effective Erythropoiesis:Module ObjectivesAnemias of Effective Erythropoiesis:Module ObjectivesAnemias of Effective Erythropoiesis:Module ObjectivesAnemias of Effective Erythropoiesis:Module ObjectivesAnemias of Effective Erythrop
4、oiesis:Module ObjectivesAnemias of Effective Erythropoiesis:Module ObjectivesAnemias of Effective Erythropoiesis:Module ObjectivesClassificationHemolytic AnemiaSites of HemolysisHemolytic AnemiaSites of HemolysisSites of HemolysisSites of HemolysisCatabolismPrimary Laboratory InvestigationPrimary La
5、boratory InvestigationMorphologyPathophysiologyClinical RelevanceRed Blood Cell MorphologyInter-relationship TriadMorphology in HASecondary Laboratory InvestigationSecondary Laboratory InvestigationSecondary Laboratory InvestigationSecondary Laboratory InvestigationSurvey of HA by TypeHereditary HAR
6、BC Membrane StructureRBC Membrane Structure+PlasmaIntegralProteinsPeripheral ProteinsLipid Bi-Layer Lipid Bi-LayerRBC CytoplasmMembrane AbnormalitiesMembrane AbnormalitiesExcess membrane cholesterolRBC inclusionsSenescent RBCComplement coated RBCAntibody coated RBCRigid RBCSpleen:Effects on RBCsSple
7、en:Effects on RBCsHereditary SpherocytosisHereditary SpherocytosisSpherocyte Morphology&Spherocyte Morphology&PathophysiologyPathophysiologyHereditary ElliptocytosisEllipto/Ovalocyte Clinical Ellipto/Ovalocyte Clinical RelevanceRelevanceHereditary ElliptocytosisHereditary ElliptocytosisHereditary El
8、liptocytosisSlides not shown.See Figure 8-7,p.91,Clinical HematologyClinical HematologyHereditary PyropoikilocytosisHereditary PyropoikilocytosisSlides not shown.See Figure 8-15,p.95,Clinical HematologyClinical HematologyHereditary StomatocytosisStomatocyte Morphology&Stomatocyte Morphology&Pathophy
9、siologyPathophysiologyHereditary StomatocytosisEnzymopathiesHemolytic AnemiaEnzymopathiesHemolytic AnemiaEnzymopathiesHemolytic AnemiaBite CellsBite CellsMorphology&PathophysiologyMorphology&PathophysiologyEnzymopathiesHemolytic AnemiaSlides not shown.See Figure 17-6,p.262,Clinical HematologyClinica
10、l HematologyEnzymopathiesHemolytic AnemiaPlasma Constituent AbnormalitiesTarget Cell(Codocyte)Target Cell(Codocyte)PathophysiologyPathophysiologyTarget Cell(Codocyte)Target Cell(Codocyte)Slides not shown.See Figure 8-11,p.92,Clinical HematologyClinical HematologyEchinocyte MorphologyEchinocyte Morph
11、ologyAcanthocyte MorphologyAcanthocyte MorphologyAcquired Hemolytic AnemiaExtracorpuscular DefectsDirection ofblood flowFibrinSchistocyteSchistocyteMicroangiopathic TraumaMicroangiopathic TraumaSchistocyteSchistocyteMicroangiopathic TraumaMicroangiopathic TraumaSlides not shown.See Figure 8-12,p.94,
12、Clinical HematologyClinical HematologyIntracorpuscular-PNHIntracorpuscular-PNHModule Objectives:Blood Loss AnemiaModule Objectives:Blood Loss AnemiaLaboratory FindingsBlood Loss AnemiaLaboratory FindingsBlood Loss AnemiaLaboratory FindingsBlood Loss AnemiaLaboratory FindingsBlood Loss AnemiaLaborato
13、ry FindingsBlood Loss AnemiaLaboratory FindingsBlood Loss AnemiaLaboratory FindingsBlood Loss AnemiaLaboratory FindingsBlood Loss AnemiaLaboratory FindingsBlood Loss AnemiaEtiologyAcute Blood Loss AnemiaEtiology-Chronic Blood Loss AnemiaPathophysiology-Chronic Blood Loss AnemiaLaboratory Investigation in Chronic Blood Loss AnemiaSession SummaryWWW Sites of InterestWWW Sites of Interest
