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Measurement of Heme Synthesis Levels in Mammalian Cells
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Iron overload complicating sideroblastic anemia--is the gene for hemochromatosis responsible?

R Barron1, N D Grace, G Sherwood

  • 1Department of Medicine, Faulkner Hospital, Boston, Massachusetts.

Gastroenterology
|April 1, 1989
PubMed

Idiopathic hemochromatosis is a hereditary disease that is associated with human leucocytic antigens A3, B7, and B14. A genetic association between human leucocytic antigen-linked hemochromatosis and idiopathic refractory sideroblastic anemia has been suggested that may predispose some patients with idiopathic refractory sideroblastic anemia to develop gross iron overload. Study of the family of a patient with idiopathic refractory sideroblastic anemia and hemochromatosis revealed that 2 of 5 first-degree relatives had significant elevations of serum ferritin, and a shared human leucocytic antigen haplotype, supporting the concept that patients with idiopathic refractory sideroblastic anemia and significant iron overload have at least one allele for hemochromatosis.

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