{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T04:17:06Z","timestamp":1791346626044,"version":"4.3.1"},"reference-count":59,"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":4150,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1995,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In Africa, the \u03b2\u2010globin gene cluster haplotype may be associated with variation of Hb F levels in subjects with sickle cell anemia (SS). These observations have not yet been conclusively confirmed in SS out of Africa, perhaps because of small sample sizes, the predominance of haplotype heterozygotes, and diverse influences, including gender, upon Hb F levels.<\/jats:p><jats:p>We studied 384 adult African\u2010American SS patients (mean age, 31 years) and explored the relationship of gender, \u03b2\u2010globin gene cluster haplotype, and \u03b1 thalassemia to hematological values and Hb F levels. Both haplotype and gender influenced Hb F concentration. In the total sample, Hb F was higher in females than in males (8.2 vs. 6.5%). In 35 males who were either homozygous for the Senegal chromosome or had the Senegal\/Benin haplotype, the mean percent Hb F (8.0%) was equivalent to the Hb F level in females with Benin and Bantu haplotypes (\u20107.5%). Both females and males homozygous for the Senegal haplotype chromosome or with the Senegal\/Benin combination had a significant increase in Hb F compared to other groups. In 44 Senegal\/Senegal or Senegal\/Benin females the Hb F was 10.9%, or 1.0 g\/dl, the highest value observed in all primary analysis groups. Preliminary analyses suggested that the presence of a Bantu chromosome blunted the gender\u2010associated difference in Hb F, but Hb F differences between females with the Senegal\/Benin haplotype (11.2%) and the Senegal\/Bantu haplotype (8.8%) were not statistically significant. Hemoglobin concentrations were higher in males than in females except in subjects with at least one Senegal haplotype chromosome, where hemoglobin levels were equal. As expected, \u03b1 thalassemia reduced the MCV, increased hemoglobin concentration, and lowered reticulocyte counts, regardless of haplotype. Hb F levels were not affected by the presence of \u03b1 thalassemia in any group.<\/jats:p><jats:p>We conclude that gender and \u03b2\u2010globin gene cluster haplotype interact significantly in the modulation of Hb F and anemia in adults with SS. \u00a91995 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.2830480307","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T13:56:18Z","timestamp":1172066178000},"page":"175-181","source":"Crossref","is-referenced-by-count":98,"title":["Gender and haplotype effects upon hematological manifestations of adult sickle cell anemia"],"prefix":"10.1002","volume":"48","author":[{"given":"M. H.","family":"Steinberg","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Hsu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. 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