Thalassemia: the continued challenge

This is a time of therapeutic hope and promise in the management of thalassemia. I began my efforts in this fascinating field 45 years ago when I saw my first patient with thalassemia intermedia. No one on the house or attending staffs believed the diagnosis. They thought this near 70 year old man, with jaundice, anemia, and intrathoracic and para spinous masses must have metastatic cancer. But his red cells were incredibly misshapen. Later, I found the telltale inclusions in them and became convinced that he had thalassemia. His case and others like his brought me to the realization that the pathophysiology of β and α thalassemia is directly related to the degree of imbalance of the synthesis of α and β chains of hemoglobin.1 I wish I had been the first to make that suggestion.