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HEMOGLOBINOPATHIES - Coggle Diagram

- - - - Hgb C polymers form a short, thick crystal within the RBC
        -Hgb C crystals do not change do not change the shape of the red cell
        -Vaso-occlusion does not occur
        
        Normo-, normo- anemia
        Some microcytosis and mild hypochromia
        Negative hemoglobin solubility test
        
        Cellulose acetate electrophoresis shows no Hgb A & shows Hgb C (co-migrating w/ A2, E, and O)
  - - - Electrophoresis shows about 60% Hgb A and 30% Hgb C
        However, C will migrate as separate band on citrate agar at an acid pH
        *On cellulose acetate, CEOA2 comigrate
  - - - Positive solubility test due to Hgb S
        On cellulose acetate electrophoresis, Hgb C & Hgb S ~ equal amounts
        
        Typically see many target cells, folded or "pocketbook" cells
        
        When crisis occurs, supportive therapy is the same as for SS pts.
        Normal life span (60-70 yrs) but can have diminished quality of life (retinal lesions)
- - - - Sickle Cell Crises- crises occurs on top of ongoing low level hemolysis in SCD (About 80% SCD pts. have only a rare crisis)
        
        Dactylitis-due to painful extramedullary hematopoieis (usually the first clinical finding in infants w/ severe SCD)
      - Shows 80-90% Hbg S remainder is usually F & A2
  - - - 30-45% Hgb S on Hgb electrophoresis
        Positive solubility test
        Normal RBC morphology except for a few targets
- - - - Brownish blood due to chronic cyanosis
        No treatment available --> Compensatory erythrocytosis (condition to compensate for chronically decreased oxygen delivery)
- - - - Homozygous state (>90% Hgb E) presents as micro-, normo- anemia with many target cells
        Can occur in combination in beta-thalassemia
- - - - Cellulose acetate electrophoresis shows 95% Hgb D which migrates to same position as S
        *Requires High Pressure Liquid Chromatography