{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T00:39:40Z","timestamp":1791247180947,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2014,1,30]],"date-time":"2014-01-30T00:00:00Z","timestamp":1391040000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2014,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hydroxyurea (<jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content>) reduces vaso\u2010occlusive crises (<jats:styled-content style=\"fixed-case\">VOC<\/jats:styled-content>) and other complications of sickle cell anaemia (<jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>). Alpha\u2010thalassaemia is a known modifier of <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>. Studies on the efficacy of <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content> in <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content> patients with \u03b1\u2010thalassaemia have yielded varying results.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To determine the effect of \u03b1\u2010thalassaemia in response to <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content> therapy in the <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>ulticenter <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>tudy of <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>ydroxyurea (<jats:styled-content style=\"fixed-case\">MSH<\/jats:styled-content>) cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We compared the laboratory parameters and <jats:styled-content style=\"fixed-case\">VOC<\/jats:styled-content> incidence in the <jats:styled-content style=\"fixed-case\">MSH<\/jats:styled-content> cohort stratified by the presence or the absence of \u03b1\u2010thalassaemia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Hydroxyurea showed significant (<jats:italic><jats:styled-content style=\"fixed-case\">P<\/jats:styled-content><\/jats:italic>\u00a0=\u00a00.001 for all baseline vs. follow\u2010up comparisons) treatment effect on red cell indices irrespective of \u03b1\u2010globin gene deletion. The magnitude of the <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content>\u2010related changes was similar for mean corpuscular volume (<jats:styled-content style=\"fixed-case\">MCV<\/jats:styled-content>) (no \u03b1\u2010thalassaemia 13\u00a0fl and \u03b1\u2010thalassaemia 13\u00a0fl) and mean corpuscular haemoglobin (<jats:styled-content style=\"fixed-case\">MCH<\/jats:styled-content>) (no \u03b1\u2010thalassaemia 4\u00a0pg and \u03b1\u2010thalassaemia 4\u00a0pg) in both groups. Foetal haemoglobin (<jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>b<jats:styled-content style=\"fixed-case\">F<\/jats:styled-content>) and <jats:styled-content style=\"fixed-case\">F<\/jats:styled-content>\u2010cells also increased significantly with <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content> treatment in both groups. Total haemoglobin increased after <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content> treatment in both groups, but the increase was smaller and not statistically significant in patients with \u03b1\u2010thalassaemia. In contrast, <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content>\u2010related reduction in <jats:styled-content style=\"fixed-case\">VOC<\/jats:styled-content>s was more pronounced in patients with \u03b1\u2010thalassaemia (<jats:styled-content style=\"fixed-case\">VOC<\/jats:styled-content> incidence rate ratio <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content>\/placebo: 0.63 for \u03b1\u2010thalassaemia and 0.54 for no \u03b1\u2010thalassaemia (<jats:italic><jats:styled-content style=\"fixed-case\">P<\/jats:styled-content><\/jats:italic> for interaction 0.003).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Hydroxyurea decreases <jats:styled-content style=\"fixed-case\">VOC<\/jats:styled-content>s in <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content> patients with and without \u03b1\u2010thalassaemia, and the degree of <jats:styled-content style=\"fixed-case\">VOC<\/jats:styled-content> reduction was more pronounced in the patients with alpha\u2010thalassaemia. Despite the lower baseline values, changes in standard laboratory parameters such as <jats:styled-content style=\"fixed-case\">MCV<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>b<jats:styled-content style=\"fixed-case\">F<\/jats:styled-content> percent remain useful in monitoring <jats:styled-content style=\"fixed-case\">HU<\/jats:styled-content> therapy in the presence of \u03b1\u2010thalassaemia.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.12245","type":"journal-article","created":{"date-parts":[[2013,12,14]],"date-time":"2013-12-14T15:18:57Z","timestamp":1387034337000},"page":"341-345","source":"Crossref","is-referenced-by-count":19,"title":["Alpha\u2010thalassaemia and response to hydroxyurea in sickle cell anaemia"],"prefix":"10.1111","volume":"92","author":[{"given":"Deepika S.","family":"Darbari","sequence":"first","affiliation":[{"name":"Division of Haematology Center for Cancer and Blood Disorders Children's National Medical Center Washington DC USA"},{"name":"Department of Paediatrics The George Washington University Medical Center Washington DC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mehdi","family":"Nouraie","sequence":"additional","affiliation":[{"name":"Howard University Center for Sickle Cell Disease Washington DC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James G.","family":"Taylor","sequence":"additional","affiliation":[{"name":"National Heart Lung and Blood Institute Vascular Medicine Branch National Institutes of Health Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo","family":"Brugnara","sequence":"additional","affiliation":[{"name":"Department of Pathology Harvard Medical School Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oswaldo","family":"Castro","sequence":"additional","affiliation":[{"name":"Howard University Center for Sickle Cell Disease Washington DC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samir K.","family":"Ballas","sequence":"additional","affiliation":[{"name":"Thomas Jefferson University Cardeza Foundation Philadelphia PA 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