Thalassemia

Abstract

IN 1966 Dr. Paul Marks¹ reviewed our understanding of the thalassemia problem in this series of Seminars. I will attempt to continue the story from where his excellent summary concluded.Unbalanced Globin Synthesis and the Pathophysiology of ThalassemiaThalassemia is a disorder of hemoglobin synthesis in which decreased amounts of hemoglobin collect in the maturing red cell. The thalassemia syndromes² are therefore classified among the hypochromic anemias. However, decreased cellular hemoglobin content is a tolerable condition because the normal marrow is capable of generating an increased number of red cells if iron stores are maintained. In thalassemia the hypochromic anemia . . .