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MLAB 1415-Hematology Keri Brophy-Martinez

MLAB 1415-Hematology Keri Brophy-Martinez. Chapter 14: Introduction to Hemolytic Anemias. Hemolytic anemia. Anemia caused by hemolysis of red blood cells Hemolysis is the destruction of erythrocytes resulting in the release of hemoglobin Results in reduction of normal red cell lifespan.

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MLAB 1415-Hematology Keri Brophy-Martinez

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  1. MLAB 1415-HematologyKeri Brophy-Martinez Chapter 14: Introduction to Hemolytic Anemias

  2. Hemolytic anemia • Anemia caused by hemolysis of red blood cells • Hemolysis is the destruction of erythrocytes resulting in the release of hemoglobin • Results in reduction of normal red cell lifespan. • Normocytic, normochromic anemia • RBC’s are prematurely destroyed

  3. Classified by… • Source of defect causing the hemolysis • Intrinsic/extrinsic • Poikilocyte present • Schistocytes/Spherocytes • Mode of onset • Inherited/acquired • Location of hemolysis • Intravascular/Extravascular

  4. Lab Features • Reticulocytosis • RPI> 2 • Erythroid hyperplasia of bone marrow • M:E ratio decreased • Peripheral blood • Normochromic,normocytic • Polychromasia • nRBCs • Tests for heme catabolism abnormal • Unconjugated/conjugated bilirubin • Haptoglobin

  5. Lab Features

  6. Diagnostic Approach to Hemolytic Anemias Increased RBC Production Increased RBC Destruction COOMBS (DAT) test Peripheral blood smear RBC Morphology Immune Hemolytic Anemias Lab Investigation Definitive Diagnosis

  7. Clinical Findings • Jaundice • Pallor • Fatigue • Cardiac symptoms • Gallstones • Dark or red urine • Splenomegaly

  8. Sites of Destruction • Intravascular • Hemolysis occurs within the circulation • RBC’s are severely damaged • Extravascular • Hemolysis occurs within the macrophages of the spleen, liver or bone marrow • More common than intravascular

  9. Intravascular Destruction • RBC is hemolyzed • Free hgb released into plasma • Hgb binds to haptoglobin • If haptoglobin depleted, hemopexin can step in • This complex goes to liver • Complex converted to bilirubin • Bilirubin excreted to intestinal tract

  10. Terms • Hemoglobinemia • Occurs if hemopexin and haptoglobin is depleted. Free hgb circulates in blood. • Hemoglobinuria • Occurs if free hgb can not be absorbed by the liver and kidney 3. Hemosiderinuria • Hemosiderin in the urine, sign of filtration of hemoglobin thru the kidney

  11. Causes ofIntravascular Hemolysis

  12. Extravascular Destruction • Hgb is degraded within the phagocyte to heme and globin • Phagocyte locations: • Spleen: removes slightly damaged RBCs • Liver: removes severely damaged RBCs • Bone marrow: remove mature precursor cells that are intrinsically abnormal • Heme further degraded to iron, biliverdin and carbon monoxide • Biliverdin enters plasma as bilirubin • Bilirubin binds to albumin, excreted by the liver

  13. Causes of Extravascular Hemolysis

  14. Source of Defect of RBC • Intrinsic • Abnormality of the RBC • Membrane • Cell enzymes • Hemoglobin molecule • Usually hereditary • EXCEPTION: Acquired • Paroxysymal noctural hemoglobinuria (PNH) • Site of hemolysis: extravascular

  15. Source of Defect of RBC • Extrinsic • Antagonist in cell’s environment causes injury to the RBC • Antagonistic plasma factors • Traumatic physical cell injury • Immune mediated cell destruction • RBC is normal • Usually acquired • Site of hemolysis: intra or extravascular

  16. Referenes • Harmening, D. M. (2009). Clinical Hematology and Fundamentals of hemostasis (5th ed.). Philadelphia, PA: F.A. Davis Company. • McKenzie, S. B. (2010). Clinical Laboratory Hematology (2nd ed.). Upper Saddle River, NJ: Pearson Education, Inc..

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