X-Linked, Pyridoxine-Responsive Sideroblastic Anemia

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Abstract
In a normal adult, 2 × 1011 worn-out red cells die every day. In replacing them with an equal number of fresh erythrocytes, the bone marrow synthesizes 4 × 1014 molecules of hemoglobin per second. These hemoglobin molecules consist of two equal partners: heme and globin. To make heme, an atom of iron is inserted from the young erythroblast into the center of an intensely colored ring of protoporphyrin IX; simultaneously, the developing red cell synthesizes two kinds of globin chains, α and β, which are joined to the heme molecule. The developing red cells coordinate these components of hemoglobin . . .