Hemolysis and Hemolytic Anemias Dr. Sabir
Hemolytic Anemias-Introduction <ul><li>Definition :  a group of disorders characterized by  decreased red cell lifespan. <...
General Clinical Features <ul><li>Splenomegaly  is generally present </li></ul><ul><li>Increased incidence of  pigmented g...
Hemolytic Anemias - Classification Schemes <ul><li>Classification by  sites of red cell destruction </li></ul><ul><li>Clas...
Hemolytic Anemias -  Sites of Red Cell Destruction <ul><li>Extravascular Hemolysis -   </li></ul><ul><ul><li>Macrophages i...
Laboratory Evidence for Presence of Hemolysis <ul><li>Evidence for  increased red cell production </li></ul><ul><li>Eviden...
Evidence for increased red cell production <ul><li>In the blood:  </li></ul><ul><ul><li>Elevated reticulocyte count </li><...
Polychromatophilic cell  on Wright-Giemsa staining Reticulocytes (supravital staining)
Erythroid Hyperplasia <ul><li>Expansion of the erythroid lineage. </li></ul><ul><li>Arrow is pointing to red cell precurso...
Frontal Bossing Results from increased erythropoietic activity and marrow space expansion
Evidence for Increased Red Cell Destruction <ul><li>Biochemical consequences of hemolysis in general </li></ul><ul><li>Mor...
Biochemical consequences of hemolysis in general <ul><li>Elevated  LDH levels </li></ul><ul><li>Elevated  unconjugated bil...
Biochemical Consequences of  Intravascular  Hemolysis <ul><li>Reduced serum  haptoglobin </li></ul><ul><li>Hemoglobinemia ...
Hemolytic Anemias - Classification by Etiology <ul><li>Congenital </li></ul><ul><ul><li>Defects in membrane skeleton prote...
Congenital Hemolytic Anemias- Membrane Defects <ul><li>Hereditary spherocytosis  is the most common defect leading to anem...
Hereditary Spherocytosis <ul><li>Defect is in proteins of the membrane skeleton, usually  spectrin  or  ankyrin </li></ul>...
Hereditary Spherocytosis <ul><li>Autosomal Dominant  trait </li></ul><ul><li>Patients develop hemolysis and abdominal pain...
HEREDITARY SPHEROCYTOSIS Osmotic Fragility
Osmotic Fragility Normal Abnormal- HS cells lyse more readily at low ionic strength
Congenital Hemolytic Anemias Enzyme Defects
Red Cell Enzyme Defects <ul><li>RBCs require constant energy to maintain biconcave disc shape and hemoglobin in reduced fo...
RBC Metabolism Simplified  Glucose Anaerobic glycolysis Aerobic metabolism Glucose-6-P G6PD <ul><li>Detoxification of meta...
G6PD Deficiency Pathophysiology <ul><li>Low G6PD activity results in low levels of NADPH and reduced glutathione, which ar...
G6PD <ul><li>Two normal forms of enzyme.  Most prevalent type is  B .  20% of healthy Africans have type  A . </li></ul><u...
G6PD Deficiency  Agents to avoid <ul><li>Primaquine </li></ul><ul><li>Aspirin </li></ul><ul><li>Quinolones(cipro) </li></u...
Favism <ul><li>Hemolysis after hours/days of ingestion of fava beans </li></ul><ul><li>Beans contain oxidants vicine & con...
G6PD Deficiency Clinical Features <ul><li>Typically, hemolysis is triggered by drugs or infections. </li></ul><ul><li>Anem...
Treatment <ul><li>Avoid oxidant drugs </li></ul><ul><li>Transfuse in severe cases </li></ul><ul><li>IV fluids to maintain ...
Congenital Hemolytic Anemias Hemoglobinopathies <ul><li>Structural Hemoglobin Defects </li></ul><ul><ul><li>Hemoglobin S <...
Acquired Hemolytic Anemias Immune-Mediated Hemolysis
Pathophysiology <ul><li>RBCs react with autoAb±complement> premature destruction of RBCs by RE system </li></ul><ul><li>RB...
Immune hemolytic anemias - Classification <ul><li>Autoimmune </li></ul><ul><ul><li>Warm antibody-mediated </li></ul></ul><...
Auto-Immune Hemolytic Anemias <ul><li>Antibodies causing hemolysis can be broken down into 2 general categories:  warm and...
Coomb’s Test <ul><li>The  Direct Coomb’s  =  DAT  (Direct Antiglobulin Test) - tests for IgG or C3  DIRECTLY ON THE RED CE...
Direct Coomb’s Test Patient’s RBCs coated with IgG or C3 Antibodies to human IgG/C3 (Coomb’s reagent) Agglutinationin the ...
Indirect Coomb’s Test Agglutinationin the test tube Patient serum containing anti RBC Abs Normal RBCs and Coomb’s reagent
Warm-Antibody Hemolytic Anemias <ul><li>Idiopathic ( most of cases) </li></ul><ul><li>2º to lymphoid malignancies (CLL, NH...
Spherocyte Formation Progressive loss of membrane, loss of surface area, more spherocytic shape
Warm-Antibody Hemolytic Anemias Diagnosis <ul><li>Spherocytosis </li></ul><ul><li>Reticulocytosis </li></ul><ul><li>Coomb’...
Warm Autoimmune Hemolytic Anemia - spherocytes
Warm-Antibody Hemolytic Anemias Treatment <ul><li>Patients may require red cell transfusions, if they are symptomatic with...
Warm-Antibody Hemolytic Anemias Immunosuppressive Treatment <ul><li>First line is  corticosteroids ( prednisone 1mg/kg/d t...
Cold Agglutinin Disease <ul><li>Pathogenic antibodies are usually  IgM , which bind to red cells in the cooler extremities...
Cold Agglutinin Disease <ul><li>In the cold, IgM can lead to red cell agglutination </li></ul><ul><li>Red cells clumps can...
Digital ischemia from cold-agglutinin disease
Cold Agglutinin Disease Clinical features <ul><li>Can be associated with infection with either  Mycoplasma or Mononucleosi...
Paroxysmal Nocturnal Hemoglobinuria <ul><li>An  acquired  disease in which an abnormal stem cell clone gives rise to abnor...
Paroxysmal Nocturnal Hemoglobinuria - Clinical features <ul><li>Pancytopenia   </li></ul><ul><li>Predisposition to thrombo...
Non-Immune Hemolytic Anemia Classification <ul><li>Mechanical trauma to red cells </li></ul><ul><ul><li>Microangiopathic H...
Microangiopathic Hemolytic Anemias <ul><li>Shear damage to red cells as the result of endothelial cell activation/damage. ...
MAHA - Schistocytes
Macroangiopathic Hemolytic Anemias <ul><li>Abnormalities within heart and large vessels can cause shear damage to RBCs. </...
March Hemoglobinuria <ul><li>1861 - German soldier complained of dark urine after strenuous marches. </li></ul><ul><li>196...
Hemolysis from Infections <ul><li>Malaria </li></ul><ul><li>Bartonella bacilliformis  </li></ul><ul><li>Clostridium welchi...
Hemolysis Associated with Chemical and Physical Agents <ul><li>Arsenic, especially arsine gas </li></ul><ul><li>Lead  </li...
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Medicine 5th year, 8th lecture/part one (Dr. Sabir)

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Medicine 5th year, 8th lecture/part one (Dr. Sabir)

  1. 1. Hemolysis and Hemolytic Anemias Dr. Sabir
  2. 2. Hemolytic Anemias-Introduction <ul><li>Definition : a group of disorders characterized by decreased red cell lifespan. </li></ul><ul><li>Normal red cell lifespan is 120d. </li></ul><ul><li>The patient may not always be anemic, depending on the degree of marrow compensation. </li></ul>
  3. 3. General Clinical Features <ul><li>Splenomegaly is generally present </li></ul><ul><li>Increased incidence of pigmented gallstones. </li></ul><ul><li>Dark urine (tea-colored or red) </li></ul><ul><li>Patients may have chronic ankle ulcers . </li></ul><ul><li>Aplastic crises associated with Parvovirus B19, may occur </li></ul><ul><li>Increased requirement for folate </li></ul>
  4. 4. Hemolytic Anemias - Classification Schemes <ul><li>Classification by sites of red cell destruction </li></ul><ul><li>Classification as acquired vs congenital </li></ul><ul><li>Classification by mechanism of red cell damage </li></ul>
  5. 5. Hemolytic Anemias - Sites of Red Cell Destruction <ul><li>Extravascular Hemolysis - </li></ul><ul><ul><li>Macrophages in spleen, liver, and marrow remove damaged or antibody-coated red cells </li></ul></ul><ul><li>Intravascular Hemolysis </li></ul><ul><ul><li>Red cells rupture within the vasculature, releasing free hemoglobin into the circulation </li></ul></ul>
  6. 6. Laboratory Evidence for Presence of Hemolysis <ul><li>Evidence for increased red cell production </li></ul><ul><li>Evidence for increased red cell destruction </li></ul>
  7. 7. Evidence for increased red cell production <ul><li>In the blood: </li></ul><ul><ul><li>Elevated reticulocyte count </li></ul></ul><ul><ul><li>Circulating NRBCs may be present </li></ul></ul><ul><li>In the bone marrow: </li></ul><ul><ul><li>erythroid hyperplasia </li></ul></ul><ul><ul><li>reduced M/E (myeloid/erythroid) ratio </li></ul></ul><ul><li>In the bone: </li></ul><ul><ul><li>Deformities in the skull and long bones (“ frontal bossing ”) </li></ul></ul>
  8. 8. Polychromatophilic cell on Wright-Giemsa staining Reticulocytes (supravital staining)
  9. 9. Erythroid Hyperplasia <ul><li>Expansion of the erythroid lineage. </li></ul><ul><li>Arrow is pointing to red cell precursor. </li></ul><ul><li>Myeloid:erythroid ratio is normally 3:1. The slide above has ratio of 1:10 . </li></ul>
  10. 10. Frontal Bossing Results from increased erythropoietic activity and marrow space expansion
  11. 11. Evidence for Increased Red Cell Destruction <ul><li>Biochemical consequences of hemolysis in general </li></ul><ul><li>Morphologic evidence of red cell damage </li></ul><ul><ul><li>seen on peripheral smear </li></ul></ul><ul><li>Reduced red cell life-span </li></ul><ul><ul><li>Measurement of red cell survival no longer routinely done. </li></ul></ul>
  12. 12. Biochemical consequences of hemolysis in general <ul><li>Elevated LDH levels </li></ul><ul><li>Elevated unconjugated bilirubin </li></ul><ul><ul><li>Only clinically significant if liver function is abnormal </li></ul></ul><ul><ul><li>Only begins to rise when red cell life span is 50 days or less </li></ul></ul>
  13. 13. Biochemical Consequences of Intravascular Hemolysis <ul><li>Reduced serum haptoglobin </li></ul><ul><li>Hemoglobinemia - red plasma </li></ul><ul><li>Hemoglobinuria - red urine </li></ul><ul><li>Hemosiderinuria - stain for iron inside cells in urine </li></ul>
  14. 14. Hemolytic Anemias - Classification by Etiology <ul><li>Congenital </li></ul><ul><ul><li>Defects in membrane skeleton proteins </li></ul></ul><ul><ul><li>Defects in enzymes involved in energy production </li></ul></ul><ul><ul><li>Hemoglobin defects </li></ul></ul><ul><li>Acquired </li></ul><ul><ul><li>Immune-mediated </li></ul></ul><ul><ul><li>Non-immune-mediated </li></ul></ul>
  15. 15. Congenital Hemolytic Anemias- Membrane Defects <ul><li>Hereditary spherocytosis is the most common defect leading to anemia, affecting 1/5000 Europeans </li></ul><ul><li>Other </li></ul><ul><ul><li>Hereditary elliptocytosis </li></ul></ul><ul><ul><ul><li>Usually no anemia </li></ul></ul></ul><ul><ul><ul><li>Autosomal dominant, affecting 1/2500 Europeans </li></ul></ul></ul><ul><ul><li>Hereditary pyropoikilocytosis </li></ul></ul>
  16. 16. Hereditary Spherocytosis <ul><li>Defect is in proteins of the membrane skeleton, usually spectrin or ankyrin </li></ul><ul><li>Lipid microvesicles are pinched off in the spleen and other RE organs, causing decreased MCV and spherocytic change. </li></ul>
  17. 17. Hereditary Spherocytosis <ul><li>Autosomal Dominant trait </li></ul><ul><li>Patients develop hemolysis and abdominal pain with even trivial infectious illnesses </li></ul><ul><li>Diagnose by  Osmotic Fragility </li></ul><ul><li>Aplastic crises with Parvovirus B19 </li></ul><ul><li>Treatment: </li></ul><ul><ul><li>Folate supplementation </li></ul></ul><ul><ul><li>Splenectomy will stop hemolysis, but spherocytes will persist. </li></ul></ul>
  18. 18. HEREDITARY SPHEROCYTOSIS Osmotic Fragility
  19. 19. Osmotic Fragility Normal Abnormal- HS cells lyse more readily at low ionic strength
  20. 20. Congenital Hemolytic Anemias Enzyme Defects
  21. 21. Red Cell Enzyme Defects <ul><li>RBCs require constant energy to maintain biconcave disc shape and hemoglobin in reduced form. </li></ul><ul><li>Without adequate energy, red cells lyse and/or deform. </li></ul><ul><li>Energy from glucose is derived from metabolism via anaerobic glycolysis (Embden-Myerhof pathway). There is an alternate aerobic pathway (pentose-phosphate shunt) which starts with G-6-P. Rate-limiting enzyme in this pathway is G6PD </li></ul>
  22. 22. RBC Metabolism Simplified Glucose Anaerobic glycolysis Aerobic metabolism Glucose-6-P G6PD <ul><li>Detoxification of metabolites of oxidative stress </li></ul><ul><li>Elimination of methemoglobin </li></ul>F-6-P
  23. 23. G6PD Deficiency Pathophysiology <ul><li>Low G6PD activity results in low levels of NADPH and reduced glutathione, which are required to protect hemoglobin from oxidative damage. </li></ul><ul><li>In the absence of adequate reducing ability (provided by G6PD), oxidizing agents convert hemoglobin to methemoglobin, then denature it, causing it to precipitate as Heinz bodies . </li></ul><ul><li>The spleen pinches off the Heinz body and the overlying membrane, leaving a “bite cell” or “blister cell” </li></ul>
  24. 24. G6PD <ul><li>Two normal forms of enzyme. Most prevalent type is B . 20% of healthy Africans have type A . </li></ul><ul><li>Deficiency is X-linked . </li></ul><ul><li>In Africans with G6PD deficiency, mutant protein is A-, which is unstable and loses activity as the red cell ages( 5-15% of normal activity). </li></ul><ul><li>Mediterranean variant has baseline low activity(1-3%activity). </li></ul><ul><li>In US, 10-14% of AA men affected </li></ul><ul><li>In ME, deficiency can be seen in women, since prevalence is high enough. </li></ul>
  25. 25. G6PD Deficiency Agents to avoid <ul><li>Primaquine </li></ul><ul><li>Aspirin </li></ul><ul><li>Quinolones(cipro) </li></ul><ul><li>Sulfa drugs </li></ul><ul><li>Dapsone </li></ul><ul><li>Vitamin K </li></ul><ul><li>Fava beans(favism) </li></ul><ul><li>Naphtha compounds (mothballs) </li></ul>Blister cell
  26. 26. Favism <ul><li>Hemolysis after hours/days of ingestion of fava beans </li></ul><ul><li>Beans contain oxidants vicine & convicine>free radicals>oxidise glutathione </li></ul><ul><li>Shock may develop-may be fatal </li></ul>
  27. 27. G6PD Deficiency Clinical Features <ul><li>Typically, hemolysis is triggered by drugs or infections. </li></ul><ul><li>Anemia is maximal 7-10 d after exposure. </li></ul><ul><li>Urine becomes dark associated with low back & abd pain </li></ul><ul><li>Individuals with A- begin to compensate for the anemia by making reticulocytes, despite continuation of drug. </li></ul><ul><li>Immediately after a hemolytic episode, G6PD levels in pts with A- may be normal , since the mature cells have been lysed, and only younger cells with normal G6PD levels, are present </li></ul><ul><li>Wait for 6 wk after hemolysis to do enzyme assay </li></ul>
  28. 28. Treatment <ul><li>Avoid oxidant drugs </li></ul><ul><li>Transfuse in severe cases </li></ul><ul><li>IV fluids to maintain good urine output </li></ul><ul><li>Splenectomy in severe recurrent hemolysis </li></ul><ul><li>Folic acid supplements </li></ul>
  29. 29. Congenital Hemolytic Anemias Hemoglobinopathies <ul><li>Structural Hemoglobin Defects </li></ul><ul><ul><li>Hemoglobin S </li></ul></ul><ul><ul><li>Hemoglobin C </li></ul></ul><ul><li>Thalassemias </li></ul><ul><ul><li>Alpha thalassemia </li></ul></ul><ul><ul><li>Beta thalassemia </li></ul></ul>
  30. 30. Acquired Hemolytic Anemias Immune-Mediated Hemolysis
  31. 31. Pathophysiology <ul><li>RBCs react with autoAb±complement> premature destruction of RBCs by RE system </li></ul><ul><li>RBCs opsonized by IgG, recognized by Fc receptors on RES macrophages >phagocytosis </li></ul><ul><li>If phagocytosis is incomplete > spherocytosis, but usually it is complete if complement is involved </li></ul>
  32. 32. Immune hemolytic anemias - Classification <ul><li>Autoimmune </li></ul><ul><ul><li>Warm antibody-mediated </li></ul></ul><ul><ul><li>Cold antibody-mediated </li></ul></ul><ul><ul><li>Paroxysmal Cold Hemoglobinuria </li></ul></ul><ul><li>Drug-related hemolysis </li></ul><ul><li>Hemolytic transfusion reactions </li></ul><ul><li>Hemolytic disease of the newborn </li></ul><ul><li>Paroxysmal Nocturnal Hemoglobinuria </li></ul>
  33. 33. Auto-Immune Hemolytic Anemias <ul><li>Antibodies causing hemolysis can be broken down into 2 general categories: warm and cold. </li></ul><ul><li>Warm antibodies react with RBCs best at 37° and typically do not agglutinate red cells </li></ul><ul><li>Cold antibodies typically react best at <32° and do cause RBC agglutination </li></ul><ul><li>The hallmark of this disease is a positive Coomb’s test </li></ul>
  34. 34. Coomb’s Test <ul><li>The Direct Coomb’s = DAT (Direct Antiglobulin Test) - tests for IgG or C3 DIRECTLY ON THE RED CELLS . </li></ul><ul><li>The Indirect Coomb’s - tests for IgG or C3 in the serum which react with generic normal red cells. This is also known as the antibody screen in blood-banking. </li></ul>
  35. 35. Direct Coomb’s Test Patient’s RBCs coated with IgG or C3 Antibodies to human IgG/C3 (Coomb’s reagent) Agglutinationin the test tube
  36. 36. Indirect Coomb’s Test Agglutinationin the test tube Patient serum containing anti RBC Abs Normal RBCs and Coomb’s reagent
  37. 37. Warm-Antibody Hemolytic Anemias <ul><li>Idiopathic ( most of cases) </li></ul><ul><li>2º to lymphoid malignancies (CLL, NHL) </li></ul><ul><li>2º to autoimmune dis. Eg: SLE </li></ul><ul><li>Age: usually pts > 50 yr </li></ul><ul><li>Clinically: highly variable: from asymptomatic-severely anemic </li></ul><ul><li>Mild jaundice common </li></ul><ul><li>Splenomegaly usual </li></ul>
  38. 38. Spherocyte Formation Progressive loss of membrane, loss of surface area, more spherocytic shape
  39. 39. Warm-Antibody Hemolytic Anemias Diagnosis <ul><li>Spherocytosis </li></ul><ul><li>Reticulocytosis </li></ul><ul><li>Coomb’s +ve </li></ul><ul><li>LDH î </li></ul><ul><li>Serum haptoglobin low </li></ul><ul><li>Investigate for underlying cause </li></ul>
  40. 40. Warm Autoimmune Hemolytic Anemia - spherocytes
  41. 41. Warm-Antibody Hemolytic Anemias Treatment <ul><li>Patients may require red cell transfusions, if they are symptomatic with their anemia </li></ul><ul><li>However, immunosuppression is the mainstay of therapy . </li></ul>
  42. 42. Warm-Antibody Hemolytic Anemias Immunosuppressive Treatment <ul><li>First line is corticosteroids ( prednisone 1mg/kg/d tapering after response) </li></ul><ul><li>If steroids fail to work, or if patient relapses after steroid taper, splenectomy may be necessary. IVIg can be used as adjunctive therapy. </li></ul><ul><li>Immunosuppressives such as cyclophosphamide or azathioprine may be required as third-line therapy. Rituximab has been used successfully. </li></ul><ul><li>Regular folic acid </li></ul>
  43. 43. Cold Agglutinin Disease <ul><li>Pathogenic antibodies are usually IgM , which bind to red cells in the cooler extremities, then fix complement. When red cells return to the warmer torso, IgM falls off. </li></ul><ul><li>Complement-coated red cells can be lysed directly within the vessel (intravascular hemolysis). </li></ul><ul><li>Alternatively, complement-coated red cells can be engulfed by complement receptors on macrophages within the liver (extravascular hemolysis) </li></ul>
  44. 44. Cold Agglutinin Disease <ul><li>In the cold, IgM can lead to red cell agglutination </li></ul><ul><li>Red cells clumps cannot pass through microvasculature, leading to cyanosis and ischemia in extremities. </li></ul><ul><li>Smear shows RBC agglutination. </li></ul>
  45. 45. Digital ischemia from cold-agglutinin disease
  46. 46. Cold Agglutinin Disease Clinical features <ul><li>Can be associated with infection with either Mycoplasma or Mononucleosis , </li></ul><ul><li>Can also be idiopathic or associated with a lymphoproliferative disease . </li></ul><ul><li>Treatment is to keep patient (especially the extremities) warm. Blood and IV fluids should be warmed. </li></ul><ul><li>Immunosuppression with oral chemotherapy may be required. </li></ul><ul><li>Steroids and splenectomy are usually ineffective. </li></ul>
  47. 47. Paroxysmal Nocturnal Hemoglobinuria <ul><li>An acquired disease in which an abnormal stem cell clone gives rise to abnormal RBCs, WBCs, and platelets all missing proteins which are attached to the cell surface by a GPI (glycero-phosphatidylinositol) anchor . </li></ul><ul><li>Among these proteins are CD55 and CD59 , which protect red cells from complement-mediated lysis. </li></ul><ul><li>Diagnose by flow cytometry for CD55 and CD59. </li></ul><ul><li>Former diagnostic test was Ham’s test - looking for abnormal sensitivity to addition of acidified serum. PNH cells lyse at a higher pH than normal red cells. </li></ul>
  48. 48. Paroxysmal Nocturnal Hemoglobinuria - Clinical features <ul><li>Pancytopenia </li></ul><ul><li>Predisposition to thrombosis. </li></ul><ul><ul><li>Both arterial and venous </li></ul></ul><ul><ul><li>Associated with Budd-Chiari syndrome. </li></ul></ul><ul><li>May develop during the course of aplastic anemia, either preceding or after recovery from aplasia. </li></ul><ul><li>May develop into acute leukemia </li></ul><ul><li>Hemolysis is predominantly nocturnal (when pH usually falls), usually mild. </li></ul>
  49. 49. Non-Immune Hemolytic Anemia Classification <ul><li>Mechanical trauma to red cells </li></ul><ul><ul><li>Microangiopathic Hemolytic Anemia </li></ul></ul><ul><ul><li>Abnormalities in heart and large vessels </li></ul></ul><ul><ul><li>March Hemoglobinuria </li></ul></ul><ul><li>Infections </li></ul><ul><li>Drugs, Chemicals, and Venoms </li></ul>
  50. 50. Microangiopathic Hemolytic Anemias <ul><li>Shear damage to red cells as the result of endothelial cell activation/damage. </li></ul><ul><li>Hallmark is presence of schistocytes on the peripheral smear. </li></ul><ul><li>Can be caused by the following disorders : </li></ul><ul><ul><li>TTP/HUS </li></ul></ul><ul><ul><li>DIC </li></ul></ul><ul><ul><li>Malignant hypertension </li></ul></ul><ul><ul><li>Ecclampsia, HELLP syndrome </li></ul></ul><ul><ul><li>Vasculitis </li></ul></ul><ul><ul><li>Other, including metastatic cancer, scleroderma renal crisis, solid organ transplant rejection </li></ul></ul>
  51. 51. MAHA - Schistocytes
  52. 52. Macroangiopathic Hemolytic Anemias <ul><li>Abnormalities within heart and large vessels can cause shear damage to RBCs. </li></ul><ul><li>Schistocytes </li></ul><ul><li>Prior to surgery: AS, ruptured sinus of valsalva, ruptured chordae, coarctation, aortic aneurysm and/or dissection. </li></ul><ul><li>After surgery: complement activation in bypass or dialysis tubing, after “patching” operations, after valve replacement </li></ul>
  53. 53. March Hemoglobinuria <ul><li>1861 - German soldier complained of dark urine after strenuous marches. </li></ul><ul><li>1964 - 2 track runners noted dark urine after races. Disappeared after softer shoe insoles used. </li></ul><ul><li>Mechanical damage to red cells also reported after conga drum playing, head beating, and karate exercises. </li></ul>
  54. 54. Hemolysis from Infections <ul><li>Malaria </li></ul><ul><li>Bartonella bacilliformis </li></ul><ul><li>Clostridium welchii toxin </li></ul><ul><ul><li>Hemolysis can be so severe it produces a dissociation b/w hemoglobin and hematocrit </li></ul></ul>
  55. 55. Hemolysis Associated with Chemical and Physical Agents <ul><li>Arsenic, especially arsine gas </li></ul><ul><li>Lead </li></ul><ul><li>Copper - deliberate ingestion, accumulation from dialysis fluid exposed to copper pipes, and Wilson’s disease. </li></ul><ul><li>Insect, spider (esp brown recluse), snake venoms </li></ul><ul><li>Heat/burns </li></ul>
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