Cooley anemia, or beta-thalassemia major, is a severe, inherited disease of the red blood cells. It is caused by absent or
markedly reduced production of beta globin, a component of hemoglobin, making the red blood cells smaller and shorter-lived than normal. Patients require
regular blood
transfusions, usually beginning in infancy. They must also be treated with the iron chelating drug deferoxamine, to prevent accumulation of toxic amounts of iron from the blood transfusions. With regular transfusion and chelation therapy, patients with Cooley anemia now live into their 30s and 40s, or longer. Cooley anemia occurs most commonly in people of Mediterranean, Asian, and African background. Persons with Cooley anemia have inherited two genes that produce insufficient beta globin. Persons who have one normal gene and one beta-thalassemia gene have only minor symptoms and a normal life span.