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March 12, 1982

Dx: Sickle Cell Anemia With Iron Deficiency Rx: Withhold Iron!!??

JAMA. 1982;247(10):1462-1464. doi:10.1001/jama.1982.03320350066035

Thalassemia, a quantitative hemoglobinopathy, plagues more racial groups and victims than the better-publicized qualitative hemoglobinopathy, sickle cell disease. These genetic diseases with their permutations afflict unnumbered millions the world over. The sickle hemoglobin phenotypes in descending order of their prevalence are AS, SS, SC, and S/β-thalassemia. Any physician may encounter one or another of these hemoglobinopathies among his patients.

Natta and co-workers discuss in this issue of The Journal (p 1442) the differential diagnostic problem presented by two mimetic phenotypes, homozygous S, or sickle cell anemia (SCA), and the doubly heterozygous hemoglobinopathy, S/β-thalassemia. Even more intriguing is the unusual management by these authors of withholding iron therapy in their patient with SCA who was proved conclusively to have iron deficiency, even though an increased incidence of infections may occur in such a patient.

An understanding of the current concepts of the sickling event will explain such perplexing management of proved