溶血性贫血医学课件

1、 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 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，称为溶溶血血性性疾疾病病。 红细胞自身异常所致的HA 红细胞外部异常所致的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 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 戊糖磷酸途径酶缺陷 如G6PD缺乏症等 无氧

2、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 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 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HA9、 人的价值，在招收诱惑的一瞬间被决定。2022-3-72022-3-7Monday, March 07, 202210、低头要有勇气，抬头要有低气。2022-3-72022-3-72022-3-73/7/2022 6:02:21 AM11、人总是珍惜为得到。2022-3-72022-3-72022-3-7Mar-227-

3、Mar-2212、人乱于心，不宽余请。2022-3-72022-3-72022-3-7Monday, March 07, 202213、生气是拿别人做错的事来惩罚自己。2022-3-72022-3-72022-3-72022-3-73/7/202214、抱最大的希望，作最大的努力。2022年3月7日星期一2022-3-72022-3-72022-3-715、一个人炫耀什么，说明他内心缺少什么。2022年3月2022-3-72022-3-72022-3-73/7/202216、业余生活要有意义，不要越轨。2022-3-72022-3-7March 7, 202217、一个人即使已登上顶峰，也仍要

4、自强不息。2022-3-72022-3-72022-3-72022-3-7 免疫性HA 血管性 生物因素 理化因素 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 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 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 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

5、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 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血管内溶血游离Hb升高（40mg/L）结合珠蛋白降低（3 The increased reticulocyte count is usually accompanied by peripheral smear RBC polychromasiaMorphologyPathophysiologyClinical Relevance Morphology specific to

6、 the type of HA may be present, e.g., spherocytes, elliptocytes and stomatocytes Decisions about what procedures to use for secondary, or follow-up, investigation are based on the findings of the hemogram and morphology Chemistry Bilirubin: Increased unconjugated bilirubin is indicative of hemolysis

7、 Chemistry, continued Haptoglobin Carries free plasma globin Decreased levels are indicative of hemolysis May be increased in inflammation masking hemolysis Chemistry, continued Increased free plasma hemoglobin Increased lactic dehydrogenase Urinalysis Hemoglobinuria or methemoglobinuria Hemosiderin

8、uria Urobilinogen Hereditary Acquired Membrane abnormalities Enzymopathies Plasma constituent abnormalities+PlasmaIntegralProteinsPeripheral ProteinsLipid Bi-Layer Lipid Bi-LayerRBC Cytoplasm Spherocytosis Elliptocytosis Pyropoikilocytosis Stomatocytosis See figure 17-1Excess membrane cholesterolRBC

9、 inclusionsSenescent RBCComplement coated RBCAntibody coated RBCRigid RBC Defect: RBC protein defects affecting spectrin Pathophysiology Increased Na+ permeability RBC rigidity Destruction in spleen Testing: Increased osmotic fragility Incubated osmotic fragility See figures 17-2 and 3 Lacks area of

10、 central pallorLacks area of central pallor Additional causesAdditional causes Immunologic reactionImmunologic reaction Physical traumaPhysical trauma Usually 6.5 Usually 6.5 m diameterm diameter Microspherocytes: 4 Microspherocytes: 25% of all RBCs)(25% of all RBCs) Macro-ovalocytes seen in Macro-o

11、valocytes seen in megaloblastic anemiasmegaloblastic anemias Non-specific finding in other types Non-specific finding in other types of anemia(25% of all RBCs)of anemia(30 30 short, blunt projections evenly short, blunt projections evenly distributed over the cell surfacedistributed over the cell su

12、rface A.K.A. burr cell, crenated A.K.A. burr cell, crenated RBC & sea urchin cellRBC & sea urchin cell Wet mount comparisonWet mount comparison 5-12 5-12 thornlike spicules unevenly thornlike spicules unevenly distributed over the cell surfacedistributed over the cell surface Projections may

13、 be club shaped or Projections may be club shaped or drumstick shaped (spur cells)drumstick shaped (spur cells) Extracorpuscular defects Abnormal blood vessel structure Mechanical injury Miscellaneous Intracorpuscular defects Immune processes Microangiopathic hemolytic anemia Disseminated intravascu

14、lar coagulation (DIC) Hemolytic uremic syndrome (HUS) Thrombotic thrombocytopenic purpura (TTP)Direction ofblood flowFibrinSlides not shown.See Figure 8-12, p. 94, Clinical HematologyClinical Hematology Major defect-Complement sensitive RBCs in acidified plasma environment Pathophysiology-Extravascu

15、lar lysis of affected RBC occurring during the evening hours leading to a chronic hemolytic anemia Secondary investigation Screening test-Sugar water test Increased hemolysis in PNH Definitive test-Hams acidified serum test (See Table 18-1, Clinical Hematology, p. 269.) Significant lysis in tubes co

16、ntaining acidified serum and complementAt the end of this module you should be able to Describe the natural course of acute blood loss anemia Describe the changes in laboratory test results that can be expected in acute blood loss anemia Explain the test results expected in chronic blood loss anemia

17、 Acute blood loss anemia No change in CBC parameters in first few hours 3-4 hours Hb/Hct Possible WBC count Possible Acute blood loss anemia, continued 12-24 hours Hb/Hct reflecting degree of blood loss Probable leukocytosis Possible thrombocytosis第一课件网网站 Acute blood loss anemia, continued 3-5 days

18、Possible MCV Possible reticulocyte count Possible RPI (3) See Table 20-2 Findings and course in chronic blood loss anemia are similar to iron deficiency anemia Morphology-Acute blood loss First few hours no significant changes 3-4 hours Possible left shift Possible NRBCs Morphology-Acute blood loss,

19、 continued 12-24 hours Possible left shift Possible NRBCs Platelet estimate Morphology-Acute blood loss, continued 3-5 days Possible macrocytosis Possible polychromasiaFindings and course in chronic blood loss anemia are similar to iron deficiency anemia Traumatic hemorrhage GI hemorrhage Operative

20、hemorrhage Ruptured aneurysms Obstetric complications Massive hemoptysis Excessive menses GI hemorrhage Colon carcinoma Hookworm Pathophysiology similar to iron deficiency anemia Anemia results from ineffective recycling of iron Serum ferritin levels to assess storage iron status Other iron studies

21、Stool occult blood to R/O GI bleeding Acute Blood Loss Anemia: /vpb/clinpath/vpb555/555rbc3/ (PowerPoint presentations; includes section on acute hemolytic anemia) Workup of Anemia: http:/ (Summary of test correlation for anemia; from Internal Medicine Board Review Course) An

22、emias: /classware/pathology/Krause/Anemias/Anemias.html (Overview of anemias from Dr. John Krauses Hematopathology Homepage, Tulane University-link provided)9、 人的价值，在招收诱惑的一瞬间被决定。22.3.722.3.7Monday, March 07, 202210、低头要有勇气，抬头要有低气。*3/7/2022 6:02:26 AM11、人总是珍惜为得到。22.3.7*Mar-227-Mar-2212、