부산대학교 도서관

We have previously determined that in African sickle cell anemia (SS) patients three different β-like globin gene cluster haplotypes are associated with different percent Gγ (one of the two types of non-α chains comprising hemoglobin F [HbF]), mean percent HbF, and percent dense cells. We report now that in adult New York SS patients, the presence of at least one chromosome with the Senegal haplotype is associated with higher Hb levels (1.2 g/dL higher) than is found for any other non-Senegal haplotype (P <.004). The percent reticulocytes and the serum bilirubin levels were lower in these patients. When the effect of α-gene number was analyzed by examining a sample of SS patients with concomitant α-thalassemia, the same results were obtained. Because the HbF level is significantly higher among the Senegal haplotype carriers in this sample, the inhibitory effect on sickling of this Hb variant may be one of the reasons for the haplotype effect. We conclude that the Senegal β-like globin gene cluster haplotype is associated with an amelioration of the hemolytic anemia that characterizes sickle cell disease.