Iron loading and endocrine functions in nontransfused patients with beta-thalassemia intermedia or sickle-cell thalassemia.

Body iron stores and endocrine functions were determined in eight patients with chronic hemolytic anemia who had received only small amounts of blood and no iron preparations. Four patients had beta-thalassemia intermedia (BTI) and four had sickle-cell thalassemia (SCT). Hemoglobin levels and degrees of hemolysis were similar in both groups of patients. The patients with BTI showed clear evidence of iron overload, whereas there was no evidence of iron accumulation in the patients with SCT. The three patients with BTI who had endocrinologic evaluations showed endocrine dysfunctions. Two patients with SCT had no endocrine abnormalities and the other two probably had some degree of primary hypogonadism. Iron overload in patients with thalassemia probably results from excessive intestinal iron absorption and can damage various parenchymal and endocrine organs, even in the absence of an external source of iron.