Serum ferritin in patients with hereditary spherocytosis

Serum ferritin was measured in 61 patients with autosomal dominant hereditary spherocytosis (HS), 44 splenectomized and 17 with intact spleens. In the majority (78%) the serum ferritin concentration was not elevated. Thirteen (22%) had midly elevated levels, including five splenectomized females (range 181--236 microgram/l), and eight males, two with intact spleens (range 236--436 microgram/l). The serum ferritin was not raised in 15 of the 17 non-splenectomized individuals. This group included one female who had been venesected for iron overload and further investigations have shown that both the genes for HS and haemochromatosis are present in her family. These results demonstrate that iron stores are usually normal in HS, and that prevention of iron overload alone is not an indication for splenectomy. The rare report of a patient with HS and severe iron overload may perhaps be explained by the fact that the gene frequency for haemochromatosis is common in the population.