Sickle cell syndromes: II. The sickle cell anemia-α-thalassemia syndrome

Five American black patients, ages 1 to 16 years, with the sickle cell anemia-α-thalassemia syndrome are described. Each patient had persistent microcytosis not explained by iron deficiency, and in each family the presence of α-thalassemia in combination with sickle cell trait was demonstrated in one of the parents. In one patient, in whom the diagnosis of sickle cell anemia was established at birth, an elevated level of Barts (γ 4 ) hemoglobin was also found. In these patients levels of alkali-resistant hemoglobin and reticulocyte counts were similar to those of sickle cell anemia patients of comparable age; however, stained smears of their peripheral blood rarely showed the presence of irreversibly sickled cells. No major ameliorative effect of the α-thalassemia on the clinical expression of the sickle cell disease of these patients was evident.