{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T15:29:56Z","timestamp":1791646196437,"version":"4.3.4"},"reference-count":30,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2018,10,9]],"date-time":"2018-10-09T00:00:00Z","timestamp":1539043200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NIH Multicenter Study of Hydroxyurea in Sickle Cell Anaemia","award":["Grant HL 45692"],"award-info":[{"award-number":["Grant HL 45692"]}]},{"name":"NIH Comprehensive Sickle Cell Center","award":["HL38632"],"award-info":[{"award-number":["HL38632"]}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2018,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>Determine the effect of fetal hemoglobin (HbF) and \u03b1\u2010thalassemia on red blood cell (RBC) deformability of patients with sickle\u2010cell anemia (SCA) with and without hydroxyurea (HU).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Adult patients were enrolled in the Sickle Cell Program of the Cardeza Foundation (Thomas Jefferson University) and were followed up prospectively during the period in which the Multicenter Study of Hydroxyurea (MSH) in patients with SCA was conducted. Ninety\u2010one patients did not receive HU, 20 patients were enrolled in MSH, and 10 patients were enrolled in an open\u2010label study of HU in SCA. Of the 20 patients enrolled in MSH, 11 took HU and nine took placebo. Control group included 113 normal individuals. Red blood cell deformability index (DI) was measured by ektacytometry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with SCA taking HU (n\u00a0=\u00a021) had higher DI than those taking placebo (n\u00a0=\u00a09) or who were not taking this therapy (n\u00a0=\u00a091). In patients without therapy, those with \u03b1\u2010thalassemia (n\u00a0=\u00a031) had higher DI than those without. We showed a significant positive correlation between the level of HbF and DI. SCA patients without \u03b1\u2010thalassemia and HbF &lt;10% (n\u00a0=\u00a048) had lower DI than patients with \u03b1\u2010thalassemia and HbF &lt;10% (n\u00a0=\u00a023) and patients with (n\u00a0=\u00a08) or without \u03b1\u2010thalassemia but with HbF &gt;10% (n\u00a0=\u00a012). DI measured in patients without \u03b1\u2010thalassemia and HbF &gt;10% was higher than in the three other subgroups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Elevated levels of HbF with or without HU and \u03b1\u2010thalassemia improve sickle RBC rheology, which, in turn, improve the clinical picture of SCA.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.13173","type":"journal-article","created":{"date-parts":[[2018,9,11]],"date-time":"2018-09-11T16:43:53Z","timestamp":1536684233000},"page":"798-803","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["Rheological properties of sickle erythrocytes in patients with sickle\u2010cell anemia: The effect of hydroxyurea, fetal hemoglobin, and \u03b1\u2010thalassemia"],"prefix":"10.1111","volume":"101","author":[{"given":"Samir K.","family":"Ballas","sequence":"first","affiliation":[{"name":"Department of Medicine, Cardeza Foundation for Hematologic Research, Sidney Kimmel Medical College Thomas Jefferson University Philadelphia Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9232-0268","authenticated-orcid":false,"given":"Philippe","family":"Connes","sequence":"additional","affiliation":[{"name":"Laboratoire LIBM EA7424, Equipe \u201cBiologie Vasculaire et du Globule Rouge\u201d Universit\u00e9 Claude Bernard Lyon 1 Villeurbanne France"},{"name":"Laboratoire d\u2019Excellence du Globule Rouge (LABEX GR\u2010Ex) PRES Sorbonne Paris France"},{"name":"Institut Universitaire de France (IUF) Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell 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