{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T09:47:32Z","timestamp":1791107252751,"version":"4.1.0"},"reference-count":44,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":5611,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1991,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In 113 black American adults with sickle cell anemia (HbSS), we examined nine polymorphic restriction sites, including the Xmnl site 5' to the <jats:sup>G<\/jats:sup>\u03b3 gene, to see whether haplotype is related to the level of HbF and the proportion of <jats:sup>G<\/jats:sup>\u03b3 chains or if it influences the hematological and clinical features of the disease. Seventy\u2010five percent of the patients were homozygous or heterozygous for the Benin (no. 19) or Central African Republic (Bantu, no.20) haplotypes; 13.3% were homozygous fo heterozygous for the Senegal (no.3) haplotype, while 11.5% had other genotypes. Of the subjects, 14.2% were either homozygous or heteroxygous for the Xmnl restriction site 5' to the <jats:sup>G<\/jats:sup>\u03b3 gene. We found no effect of haplotype on HbF levels. The level of <jats:sup>G<\/jats:sup>\u03b3 chains was 60.5% \u00b1 17.0% in individuals with other haplotypes. Subjects with the Xmnl site 5' to the <jats:sup>G<\/jats:sup>\u03b3 gene had <jats:sup>G<\/jats:sup>\u03b3 globin levels of 59.5% \u00b1 16.7% while those lacking that site had an average of 47.2% \u00b1 12.1%. There were no significant differences among these groups in hemoglobin concentration, packed cell volume, mean cell volume, or haplotype 3 and the 5' <jats:sup>G<\/jats:sup> \u03b3 Xmnl site were associated with increased <jats:sup>G<\/jats:sup>\u03b3 chains, there was no effect on HbF level or other hematological and clinical features that might reflect disease severity. It is likelu that determinants unrelated to haplotype, linked or unlinked to the \u03b2\u2010globin gene cluster, are the major effectors of differences in the levels of HbF in American patients with sickle cell anemia.<\/jats:p>","DOI":"10.1002\/ajh.2830360305","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T12:08:55Z","timestamp":1172059735000},"page":"184-189","source":"Crossref","is-referenced-by-count":35,"title":["Effect of \u03b2\u2010Globin gene cluster haplotype on the hematological and clinical features of sickle cell anemia"],"prefix":"10.1002","volume":"36","author":[{"given":"R. F.","family":"Rieder","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Safaya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Gillette","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Fryd","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Hsu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"III","given":"J. G.","family":"Adams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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