Thalassemia

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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 . . .