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Normocytic normochromic anaemia Hemolytic anaemia

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Hemolytic anaemia

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HEMOLYTIC ANAEMIA Dr. OM JHA
Introduction Characterized by: Premature RBC destruction (less than the normal 120-day lifespan). Elevated erythropoietin with increased erythropoiesis. Increased hemoglobin catabolites (e.g., bilirubin) Excess serum bilirubin is unconjugated. Ultimate levels of hyperbilirubinemia depend on: liver functional capacity Rate of hemolysis Normal liver severe jaundice rarely.
Contd BM erythropoiesis unable to compensate for degree of destruction of red cells Anaemia develops. Clinical and lab findings indicates: Accelerated destruction of red cells. Compensatory marrow regeneration. Mild hemolysis compensatory BM function adequate No manifestation of anaemia Compensated hemolytic anaemia.
Classification Location of hemolysis: Intravascular and extravascular. Source of defect causing hemolysis: Intracorpuscular defect and extracorpuscular defect Mode of onset: Hereditary and acquired disorders. Underlying mechanisms of hemolysis: Immune or Non-immune.
Classification
Contd..
Red Cells Destruction Extra-vascular Hemolysis Normal site of destruction of old RBCs: Spleen (80- 90%). In HA This mechanism Exaggerated. Intra-vascular Hemolysis RBCs in circulation get destroyed release of Hb in plasma. Main pathway of: PNH.
Normocytic normochromic anaemia Hemolytic anaemia
Normocytic normochromic anaemia Hemolytic anaemia
Extravascular hemolysis Occurs in macrophages of the spleen (and other organs). Predisposing factors: RBC membrane injury Reduced deformability Opsonization Principal clinical features are: Anemia, splenomegaly, and jaundice Modest reductions in haptoglobin a serum protein that binds hemoglobin
Contd Increased unconjugated bilirubin: jaundice. Increased stercobilinogen: dark colored stool. Increased urobilinogen: high colored urine. Increased iron store: iron released from heme stored in BM.
Intravascular hemolysis RBCs ruptured by: Mechanical injury (e.g., mechanical cardiac valves) Complement fixation (e.g., mismatched blood transfusion) Intracellular parasites (e.g., malaria) Extracellular toxins (e.g., clostridial enzymes). Presentation: Anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, and jaundice Markedly reduced serum haptoglobin.
Contd Characteristic findings of intra-vascular hemolysis: Hemoglobinemia Haemoglobinuria Haemosiderinuria S. Heptoglobin: decreased
Laboratory Findings Increase S. Bilirubun (Unconjugated). Increase Urine Urobilinogen. Increase rate of Bilirubin production. Increase S. LDH. Decrease life span of red cells. Decrease Heptoglobin.
Clinical Manifestations Clinical signs & symptoms depends upon: Severity of hemolysis. Duration of hemolysis. Manifestaions mostly seen in Chronic HA: Pallor Jaundice Splenomegaly Gall stones Skeletal abnormalities Leg ulcers
Compensatory Mechanisms To Hemolysis BM Erythroid hyperplasia: Chronic hemolysis --> Anaemia --> Increase in Erythropoietin --> BM erythroid hyperplasia. Reversal of M:E ratio. (2-4:1 --> 1:1-6). Reticulocytosis: BM erythroid hyperplasia --> rise in Reticulocytes. Mild: Hemoglobinopathies. Moderate to marked: IHA, HS, G6PD def.
Peripheral Blood Findings Polychromatophilia: Large red cells released from BM. Corresponds to reticulocytes. Nucleated RBC (nRBC): Seen in moderate to marked anaemia. Neutrophilia: Active moderate to marked hemolysis. Neutrophilia with shift to left (metamyelocyte, Myelocytes). Thrombocytosis: Acute hemolysis Numerous and large platelets.
Morphologic Red Cell Abnormalities Provide clue to the underlying hemolytic conditions. Spherocytes Sickle cells Target Cells Schistocytes Acanthocytes
Fig: Marrow aspirate smear from a patient with hemolytic anemia. There is an increased number of maturing erythroid progenitors (normoblasts).
Structure of RBC Membrane
RBC Membrane Defects
Hereditory Spherocytosis D/t cytoskeletal or membrane protein defects. Render RBCs spheroidal and less deformable Vulnerable to splenic sequestration and destruction. AD in 75% of patients.
Pathogenesis Insufficiency in several different proteins: spectrin, ankyrin, band 3, or band 4.2. Lead to reduced density of membrane skeletal components Reduced stability of the lipid bilayer. Loss of membrane fragments as RBCs age. Reduction in surface area RBCs assume a spheroidal shape. Diminished deformability. Propensity for being trapped and destroyed by splenic macrophages.
Normocytic normochromic anaemia Hemolytic anaemia
Fig: Reduced membrane stability in HS --> loss of RBC membrane --> formation of microspherocytes --> Ingested by splenic Macrophages.
Mechanism of Hemolysis in HS
Morphology Spherocytic RBCs are small and lack central pallor. Reticulocytosis and marrow erythroid hyperplasia. Marked splenic congestion with prominent erythrophagocytosis in the cords of Billroth.
Contd...
Clinical Features Diagnosis depends on: family history, hematologic findings, and increased RBC osmotic fragility MCHC increased: d/t cellular dehydration. Characteristic: Anemia, moderate splenomegaly, and jaundice. Clinical course: typically stable due to compensatory increases in erythropoiesis. Increased RBC turnover or diminished erythropoiesis can be problematic.
Contd... Parvovirus --> transient suppression of erythropoiesis --> Aplastic crisis Events that increase splenic RBC destruction ---> e.g., infectious mononucleosis --> trigger hemolytic crisis. 50% adults: chronic hyperbilirubinemia --> gallstones.
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Normocytic normochromic anaemia Hemolytic anaemia

  • 2. Introduction Characterized by: Premature RBC destruction (less than the normal 120-day lifespan). Elevated erythropoietin with increased erythropoiesis. Increased hemoglobin catabolites (e.g., bilirubin) Excess serum bilirubin is unconjugated. Ultimate levels of hyperbilirubinemia depend on: liver functional capacity Rate of hemolysis Normal liver severe jaundice rarely.
  • 3. Contd BM erythropoiesis unable to compensate for degree of destruction of red cells Anaemia develops. Clinical and lab findings indicates: Accelerated destruction of red cells. Compensatory marrow regeneration. Mild hemolysis compensatory BM function adequate No manifestation of anaemia Compensated hemolytic anaemia.
  • 4. Classification Location of hemolysis: Intravascular and extravascular. Source of defect causing hemolysis: Intracorpuscular defect and extracorpuscular defect Mode of onset: Hereditary and acquired disorders. Underlying mechanisms of hemolysis: Immune or Non-immune.
  • 7. Red Cells Destruction Extra-vascular Hemolysis Normal site of destruction of old RBCs: Spleen (80- 90%). In HA This mechanism Exaggerated. Intra-vascular Hemolysis RBCs in circulation get destroyed release of Hb in plasma. Main pathway of: PNH.
  • 10. Extravascular hemolysis Occurs in macrophages of the spleen (and other organs). Predisposing factors: RBC membrane injury Reduced deformability Opsonization Principal clinical features are: Anemia, splenomegaly, and jaundice Modest reductions in haptoglobin a serum protein that binds hemoglobin
  • 11. Contd Increased unconjugated bilirubin: jaundice. Increased stercobilinogen: dark colored stool. Increased urobilinogen: high colored urine. Increased iron store: iron released from heme stored in BM.
  • 12. Intravascular hemolysis RBCs ruptured by: Mechanical injury (e.g., mechanical cardiac valves) Complement fixation (e.g., mismatched blood transfusion) Intracellular parasites (e.g., malaria) Extracellular toxins (e.g., clostridial enzymes). Presentation: Anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, and jaundice Markedly reduced serum haptoglobin.
  • 13. Contd Characteristic findings of intra-vascular hemolysis: Hemoglobinemia Haemoglobinuria Haemosiderinuria S. Heptoglobin: decreased
  • 14. Laboratory Findings Increase S. Bilirubun (Unconjugated). Increase Urine Urobilinogen. Increase rate of Bilirubin production. Increase S. LDH. Decrease life span of red cells. Decrease Heptoglobin.
  • 15. Clinical Manifestations Clinical signs & symptoms depends upon: Severity of hemolysis. Duration of hemolysis. Manifestaions mostly seen in Chronic HA: Pallor Jaundice Splenomegaly Gall stones Skeletal abnormalities Leg ulcers
  • 16. Compensatory Mechanisms To Hemolysis BM Erythroid hyperplasia: Chronic hemolysis --> Anaemia --> Increase in Erythropoietin --> BM erythroid hyperplasia. Reversal of M:E ratio. (2-4:1 --> 1:1-6). Reticulocytosis: BM erythroid hyperplasia --> rise in Reticulocytes. Mild: Hemoglobinopathies. Moderate to marked: IHA, HS, G6PD def.
  • 17. Peripheral Blood Findings Polychromatophilia: Large red cells released from BM. Corresponds to reticulocytes. Nucleated RBC (nRBC): Seen in moderate to marked anaemia. Neutrophilia: Active moderate to marked hemolysis. Neutrophilia with shift to left (metamyelocyte, Myelocytes). Thrombocytosis: Acute hemolysis Numerous and large platelets.
  • 18. Morphologic Red Cell Abnormalities Provide clue to the underlying hemolytic conditions. Spherocytes Sickle cells Target Cells Schistocytes Acanthocytes
  • 19. Fig: Marrow aspirate smear from a patient with hemolytic anemia. There is an increased number of maturing erythroid progenitors (normoblasts).
  • 20. Structure of RBC Membrane
  • 22. Hereditory Spherocytosis D/t cytoskeletal or membrane protein defects. Render RBCs spheroidal and less deformable Vulnerable to splenic sequestration and destruction. AD in 75% of patients.
  • 23. Pathogenesis Insufficiency in several different proteins: spectrin, ankyrin, band 3, or band 4.2. Lead to reduced density of membrane skeletal components Reduced stability of the lipid bilayer. Loss of membrane fragments as RBCs age. Reduction in surface area RBCs assume a spheroidal shape. Diminished deformability. Propensity for being trapped and destroyed by splenic macrophages.
  • 25. Fig: Reduced membrane stability in HS --> loss of RBC membrane --> formation of microspherocytes --> Ingested by splenic Macrophages.
  • 27. Morphology Spherocytic RBCs are small and lack central pallor. Reticulocytosis and marrow erythroid hyperplasia. Marked splenic congestion with prominent erythrophagocytosis in the cords of Billroth.
  • 29. Clinical Features Diagnosis depends on: family history, hematologic findings, and increased RBC osmotic fragility MCHC increased: d/t cellular dehydration. Characteristic: Anemia, moderate splenomegaly, and jaundice. Clinical course: typically stable due to compensatory increases in erythropoiesis. Increased RBC turnover or diminished erythropoiesis can be problematic.
  • 30. Contd... Parvovirus --> transient suppression of erythropoiesis --> Aplastic crisis Events that increase splenic RBC destruction ---> e.g., infectious mononucleosis --> trigger hemolytic crisis. 50% adults: chronic hyperbilirubinemia --> gallstones.
  • 31. ???