Serum Ferritin in Patients with Hereditary Spherocytosis

Abstract

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 mildly elevated levels, including 5 splenectomized females (range 181-236 .mu.g/l), and 8 males, 2 with intact spleens (range 236-436 .mu.g/l). The serum ferritin was not raised in 15 of the 17 non-splenectomized individuals. This group included 1 female who had been venesected for Fe overload; both the genes for HS and hemochromatosis are present in her family. Fe stores are usually normal in HS. Prevention of Fe overload alone is not an indication for splenectomy. The rare report of a patient with HS and severe Fe overload may perhaps be explained by the fact that the gene frequency for hemochromatosis is common in the population.