The most severe form of beta thalassemia (sometimes called thalassemia major). Few or no functional beta globin chains are made, and this results in a life-threatening anemia. The patient requires lifelong blood transfusions, and these lead to an iron overload that requires chelation therapy. The eponym refers to Thomas Benton Cooley, an American pediatrician who first described the condition in 1925. See Desferal, thalassemia.
Subjects: Genetics and Genomics.