- Genetic abnormality (AD) leading to a quantative deficiency of either a or b chains of HbA (a2b2)
- Normally
- Two b-globin genes (one on each Chromosome 11)
- Four a-globin genes (two on each chromosome)
- b-thalassemia
- a-thalassemia
- Treatment
- Mainstay of treatment is transfusion and chelation therapy
- Stem cell transplant is playing greater role
- Splenectomy rarely required