Defining Thalassemia

Anemia in Alpha Thalassemia

  • In thalassemia there is often an excess production or accumulation of globin chains produced by genes that are not effected by the thalassemia deletion.
  • In alpha thalassemia this may be seen as gamma chain tetramers (hemoglobin Bart's) in the unborn child and as beta chain tetramers (hemoglobin H) in adults.
  • Tetramer accumulation often leads to red blood cell damage and hemolytic anemia.

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