Hemolytic Anemias: Intracorpuscular Defects

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Author:
narine010
ID:
208485
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Hemolytic Anemias: Intracorpuscular Defects
Updated:
2013-03-20 09:01:49
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Hematology anemia
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Description:
hemolytic anemias
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  1. What are the types of Hemolysis?
    • 1. Extravascular vs. intravascular sites
    • 2. Extracorpuscular vs. intracorpuscular defects
    • 3. Hereditary vs. acquired
  2. Describe the Extravascular hemolysis?
    RBC-->Heme, Globin

    Heme-->Fe, Protoporphyrin-->CO

    protoporphyrin-->unconjugated bilirubin-->liver-->conjugated bilirubin excreted__>urobilinogen and urobilin

    • conjugated bilirubin excreted Kidney__>urobilinogen and bilirubin
  3. Describe Intravascular Hemolysis?
  4. What are the Clinical Features of Hemolysis?
    • low RBC, haptoglobin
    • high Hgb breakdown, bilirubin, urobilinogen in feces and hemoglobin in urine
    • hemosiderin in kidney tubules
  5. How does the Bone Marrow respond?
    • erythroid hyperplasia
    • NRBC, increased reticulocytes, howel-jolly bodies
  6. What are the intracorpuscular Defects? (Defective RBC Membrane)
    • Hereditary spherocytosis
    • Hereditary elliptocytosis
    • hereditary stomatocytosis
    • hereditary xerocytosis
  7. What is the etiology of Spherocytosis?
    • spectrin and ankyrin deficency
    • conditioning of RBC by spleen
  8. Laboratory findings in Spherocytosis
    • hyperbilirubinemia
    • reduced haptoglobin
    • increase reticulocyte
    • MCHC increased
  9. What can you use the Osmotic Fragility test for?
    It can be used to test the integrity of cellular membrane
  10. What is the etiology of Elliptocytosis?
    • spectrin and/or protein deficiency or dysfunction?
    • membrane is weakened in stress
  11. laboratory findings of elliptocytosis?
    • slight reticulocytosis
    • decrease haptoglobin
    • normal rbc indices
  12. What is the etiology of stomatocytosis and xerocytosis? Laboratory findings?
    permeable membrane permits cation leaks into and out of RBC

    • Increased MCV
    • Decreased MCHC
    • macrocytosis
    • stomatocytes
  13. Hereditary Stomatocytosis
    • Sodium leaks into cell and K leaks out
    • H2O enters and RBC swell
  14. Hereditary xerocytosis
    H2O exits the cell causing dehydration
  15. What is G6PD deficiency?
    • lack of an enzyme that converts NADP to NADH
    • Only pentose phosphate pathway that reduces Glutathione
  16. Glutathione
    protects hemoglobin from oxidative denaturation
  17. Hgb reduction
    Methemoglobin reductase reduces methhbg to hgb using NADH 
  18. G6PD laboratory Findings
    • decreased Hgb
    • hemoglobinuria
    • elevated bilirubin
    • decreased haptoglobin
    • drug induced
  19. What is Pyruvate Kinase Deficiency?
    • decrease ATP per cell
    • leads to cell water loss and shrinkage (echinocytes)
    • increased membrane rigidity
  20. Methemoglobin Reductase Deficiency
    • cause increase levels of methemoglobin
    • not an effective hemoglobin carrier
    • may be drug induced

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