The senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients

Ronald L. Nagel, Soili Erlingsson, Mary E. Fabry, Helena Croizat, Sandra M. Susuka, Herbert M. Lachman, Millicent Sutton, Catherine Driscoll, Eric E. Bouhassira, Henny H. Billett

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)1371-1375
Number of pages5
JournalBlood
Volume77
Issue number6
StatePublished - Mar 15 1991

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Fetal Hemoglobin
Senegal
Sickle Cell Anemia
African Americans
Haplotypes
Anemia
Globins
Genes
DNA
Chromosomes
Multigene Family
Bilirubin
Thalassemia
Reticulocytes
Hemolytic Anemia
Serum

ASJC Scopus subject areas

  • Hematology

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The senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients. / Nagel, Ronald L.; Erlingsson, Soili; Fabry, Mary E.; Croizat, Helena; Susuka, Sandra M.; Lachman, Herbert M.; Sutton, Millicent; Driscoll, Catherine; Bouhassira, Eric E.; Billett, Henny H.

In: Blood, Vol. 77, No. 6, 15.03.1991, p. 1371-1375.

Research output: Contribution to journalArticle

Nagel, RL, Erlingsson, S, Fabry, ME, Croizat, H, Susuka, SM, Lachman, HM, Sutton, M, Driscoll, C, Bouhassira, EE & Billett, HH 1991, 'The senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients', Blood, vol. 77, no. 6, pp. 1371-1375.
Nagel RL, Erlingsson S, Fabry ME, Croizat H, Susuka SM, Lachman HM et al. The senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients. Blood. 1991 Mar 15;77(6):1371-1375.
Nagel, Ronald L. ; Erlingsson, Soili ; Fabry, Mary E. ; Croizat, Helena ; Susuka, Sandra M. ; Lachman, Herbert M. ; Sutton, Millicent ; Driscoll, Catherine ; Bouhassira, Eric E. ; Billett, Henny H. / The senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients. In: Blood. 1991 ; Vol. 77, No. 6. pp. 1371-1375.
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AU - Croizat, Helena

AU - Susuka, Sandra M.

AU - Lachman, Herbert M.

AU - Sutton, Millicent

AU - Driscoll, Catherine

AU - Bouhassira, Eric E.

AU - Billett, Henny H.

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N2 - 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.

AB - 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.

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