Iron stores in adults with sickle cell anemia.

Iron status was investigated in 60 adults with sickle cell anemia as determined by stainable iron in bone marrow aspirates, serum ferritin, serum iron, and the total iron-binding capacity. Seventeen patients (28%) were found to have absent bone marrow iron. There was an excellent inverse correlation between serum ferritin and the serum transferrin (p less than 0.001), and a significant positive correlation between serum ferritin and transferrin saturation (p less than 0.005), bone marrow iron (p less than (p less than 0.001), and history of prior blood transfusion (p = 0.005). Results of complete radiologic examination of the gastrointestinal tract and proctoscopy were negative in the 17 patients in the iron-deficient group. The high incidence of iron deficiency may be related to excessive urinary losses of iron. Our data indicate that serum ferritin values of less than 30 ng/ml are diagnostic of iron deficiency in patients with sickle cell anemia, with a high degree of specificity (98.7%). However the sensitivity of the test at a serum ferritin level of 30 ng/ml is only 32%. The diagnostic evaluation and the management of sickle cell anemia in iron-deficient patients needs to be better defined.