溶血性貧血醫(yī)學(xué)課件

1、 定義 臨床分類 臨床表現(xiàn) 發(fā)病機制與實驗室檢查 診斷和鑒別診斷 治療 溶血是一組由于后天或先天的各種原因使紅細胞遭破壞壽命縮短的過程。HA系指紅細胞破壞超過骨髓造血代償功能而發(fā)生的一種貧血。如溶血發(fā)生而骨髓能代償時，臨床無貧血，稱為溶溶血血性性疾疾病病。 紅細胞自身異常所致的HA 紅細胞外部異常所致的HA 紅細胞膜異常 遺傳性紅細胞酶的缺乏 遺傳性珠蛋白生成障礙 血紅素異常 遺傳性紅細胞膜缺陷 遺傳性球形紅細胞增多癥、遺傳性橢圓形紅細胞增多癥、遺傳性棘性細胞增多癥、遺傳性口形細胞增多癥 獲得性血細胞膜糖化肌醇磷酯（GPI）錨鏈膜蛋白異常，如PNH 戊糖磷酸途徑酶缺陷 如G6PD缺乏癥等 無氧

2、糖酵解途徑缺陷 如丙酮酸激酶缺乏癥等 核苷代謝酶系、氧化還原酶系等缺陷等 珠蛋白肽鏈結(jié)構(gòu)異常不穩(wěn)定血紅蛋白病、血紅蛋白病S、D、E等 珠蛋白肽鏈數(shù)量異常地中海貧血 先天性紅細胞卟啉代謝異常如紅細胞生成性血卟啉病，原卟啉型、尿卟啉型和糞卟啉型 鉛中毒可影響血紅素的合成發(fā)生HA9、 人的價值，在招收誘惑的一瞬間被決定。2022-3-72022-3-7Monday, March 07, 202210、低頭要有勇氣，抬頭要有低氣。2022-3-72022-3-72022-3-73/7/2022 6:02:21 AM11、人總是珍惜為得到。2022-3-72022-3-72022-3-7Mar-227-

3、Mar-2212、人亂于心，不寬余請。2022-3-72022-3-72022-3-7Monday, March 07, 202213、生氣是拿別人做錯的事來懲罰自己。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、一個人炫耀什么，說明他內(nèi)心缺少什么。2022年3月2022-3-72022-3-72022-3-73/7/202216、業(yè)余生活要有意義，不要越軌。2022-3-72022-3-7March 7, 202217、一個人即使已登上頂峰，也仍要

4、自強不息。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溫抗體型或冷抗體型（冷凝集型、D-L抗體型）；原發(fā)性或繼發(fā)性（SLE、病毒或藥物等0 同種免疫性HA如血型不合的輸血反應(yīng)、新生兒HA 微血管病性HA如（TTP/HUS）、DIC、敗血癥等 瓣膜病：鈣化性主動脈瓣狹窄、人工心瓣膜和血管炎等 血管壁受到反復(fù)擠壓：行軍性血紅蛋白尿 蛇毒、瘧疾和黑熱病等 大面積燒傷、血漿中滲透壓改變和化學(xué)因素如苯肼、亞硝酸鹽等中毒，可引起獲得性高鐵血紅蛋白血癥而溶血 急性溶血：起病急，突然寒戰(zhàn)、高熱、頭痛、四肢酸痛等，面色蒼白、血紅蛋白尿和

5、黃疸。嚴(yán)重者周圍循環(huán)衰竭和急性腎衰竭 慢性溶血：起病慢，常有貧血、黃疸和肝脾腫大三個特征 膽石癥、肝功能損害 嚴(yán)重溶血時骨髓腔擴大，X線骨皮質(zhì)變薄、骨骼變形 紅細胞破壞和血紅蛋白降解 紅系代償性增生 紅細胞具有缺陷或壽命縮短血管內(nèi)溶血游離Hb升高（40mg/L）結(jié)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第一課件網(wǎng)網(wǎng)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、 人的價值，在招收誘惑的一瞬間被決定。22.3.722.3.7Monday, March 07, 202210、低頭要有勇氣，抬頭要有低氣。*3/7/2022 6:02:26 AM11、人總是珍惜為得到。22.3.7*Mar-227-Mar-2212、