Nonblack patients with sickle cell disease have African beta S gene cluster haplotypes
- 26 May 1989
- journal article
- research article
- Published by American Medical Association (AMA) in JAMA
- Vol. 261 (20) , 2991-2994
- https://doi.org/10.1001/jama.261.20.2991
Abstract
Of 18 nonblack patients with sickle cell disease, 14 had sickle cell anemia, 2 had hemoglobin SC disease, and 2 had hemoglobin S-.beta..degree.-thalassemia. The .beta.s gene cluster haplotypes that were determined in 7 patients were of African origin and were identified as Central African Republic, Central African Republic minor II, Benin, and Senegal. The haplotype Central African Republic minor II was present on the .beta..degree.-thalassemia chromosome in 2 patients. None of 10 patients whose .alpha.-gene status was determined had .alpha.-thalassemia-2. These data strongly support the concept that the .beta.s gene on chromosome 11 of these individuals is of African origin and that the .alpha.-gene locus on chromosome 16 is of white or native American origin. The clinical severity of the disease in these nonblack patients is appropriate to their haplotype without .alpha.-thalassemia-2 and is comparable with that of black patients. All persons with congenital hemolytic anemia should be examined for the presence of sickle cell disease regardless of physical appearance or ethnic background.This publication has 13 references indexed in Scilit:
- HIGH FETAL HEMOGLOBIN PRODUCTION IN SICKLE-CELL-ANEMIA IN THE EASTERN PROVINCE OF SAUDI-ARABIA IS GENETICALLY-DETERMINED1986
- Hematologically and Genetically Distinct Forms of Sickle Cell Anemia in AfricaNew England Journal of Medicine, 1985
- Common haplotype dependency of high G gamma-globin gene expression and high Hb F levels in beta-thalassemia and sickle cell anemia patients.Proceedings of the National Academy of Sciences, 1985
- Evidence for the multicentric origin of the sickle cell hemoglobin gene in Africa.Proceedings of the National Academy of Sciences, 1984
- Origin of the beta S-globin gene in blacks: the contribution of recurrent mutation or gene conversion or both.Proceedings of the National Academy of Sciences, 1984
- Concurrent sickle cell anemia and alpha-thalassemia. Effect on pathological properties of sickle erythrocytes.Journal of Clinical Investigation, 1984
- Newborn diagnosis of abnormal hemoglobins from a large municipal hospital in Los Angeles.American Journal of Public Health, 1981
- Pneumococcal septicemia in children with sickle cell anemia. Changing trend of survivalJAMA, 1981
- Sickle Cell Anemia in Two White American Children: Essential Laboratory Criteria for DiagnosisPediatrics, 1979
- Sickle Cell Anemia in an American White Boy of Greek AncestryArchives of Pediatrics & Adolescent Medicine, 1977