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Major and Intermediate Syndromes of Thalassemia

Beta-thalassemia

Beta-thalassemia is characterized by:

·         Mutations in the beta globin gene

·         Severe anemia beginning from early childhood

·         It can be identified at birth by screening for sickle-cell disease

Beta-thalassemia requires:

·         Long-term transfusions

·         Iron chelation therapy

·         Hematopoietic stem cell transplantation (the only curative solution)

Alpha-thalassemia

·         More complex than beta-thalassemia

·         More widespread globally than beta-thalassemia

·         Moderate anemia, allowing patients to live a somewhat normal life

·         Little or no need for transfusions

·         Exceptional long-term transfusions

Treatment

·         Red blood cell transfusion

·         Iron chelation treatment after 10 to 20 transfusions

·         Deferiprone is indicated when DFO treatment is counter-indicated

·         Splenectomy is indicated in case of hypersplenism or for lowering transfusion requirements

Therapeutic Education

·         Learning self-infusion of medicine

·         Physical exercise

·         Adequate sun exposure

·         Smoking cessation

·         Following nutritional rules