Hemolytic Anemias: Intracorpuscular Defects
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What are the types of Hemolysis?
- 1. Extravascular vs. intravascular sites
- 2. Extracorpuscular vs. intracorpuscular defects
- 3. Hereditary vs. acquired
Describe the Extravascular hemolysis?
protoporphyrin-->unconjugated bilirubin-->liver-->conjugated bilirubin excreted__
>urobilinogen and urobilin
- conjugated bilirubin excreted Kidney__>urobilinogen and bilirubin
Describe Intravascular Hemolysis?
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
How does the Bone Marrow respond?
- erythroid hyperplasia
- NRBC, increased reticulocytes, howel-jolly bodies
What are the intracorpuscular Defects? (Defective RBC Membrane)
- Hereditary spherocytosis
- Hereditary elliptocytosis
- hereditary stomatocytosis
- hereditary xerocytosis
What is the etiology of Spherocytosis?
- spectrin and ankyrin deficency
- conditioning of RBC by spleen
Laboratory findings in Spherocytosis
- reduced haptoglobin
- increase reticulocyte
- MCHC increased
What can you use the Osmotic Fragility test for?
It can be used to test the integrity of cellular membrane
What is the etiology of Elliptocytosis?
- spectrin and/or protein deficiency or dysfunction?
- membrane is weakened in stress
laboratory findings of elliptocytosis?
- slight reticulocytosis
- decrease haptoglobin
- normal rbc indices
What is the etiology of stomatocytosis and xerocytosis? Laboratory findings?
permeable membrane permits cation leaks into and out of RBC
- Increased MCV
- Decreased MCHC
- Sodium leaks into cell and K leaks out
- H2O enters and RBC swell
H2O exits the cell causing dehydration
What is G6PD deficiency?
- lack of an enzyme that converts NADP to NADH
- Only pentose phosphate pathway that reduces Glutathione
protects hemoglobin from oxidative denaturation
Methemoglobin reductase reduces methhbg to hgb using NADH
G6PD laboratory Findings
- decreased Hgb
- elevated bilirubin
- decreased haptoglobin
- drug induced
What is Pyruvate Kinase Deficiency?
- decrease ATP per cell
- leads to cell water loss and shrinkage (echinocytes)
- increased membrane rigidity
Methemoglobin Reductase Deficiency
- cause increase levels of methemoglobin
- not an effective hemoglobin carrier
- may be drug induced
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