Mild Sickle Cell Disease

Twenty-one patients with mild sickle cell disease (MSD) between 18 and 56 years of age had mild, infrequent, or no pain crises, were active or employed, and led relatively normal lives. Mean hemoglobin concentration, reticulocyte count, and hemoglobin A2and F levels did not distinguish this group from a smaller group of patients with typical sickle cell anemia (SSA). On the basis of electrophoresis, hemoglobin H preparations, and distribution of hemoglobin F in erythrocytes, there was no evidence for thalassemia, hereditary persistence of hemoglobin F, or other hemoglobinopathies that might modify the course of SSA. There may be many host or environmental factors that protect individuals with MSD. Knowledge of these may suggest new therapeutic strategies for the control of SSA.