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Relations between red cell density (assessed with phthalate density-distribution profile method) and several hematologic, biochemical, genetic parameters, and clinical manifestations were studied in a large cohort of homozygous patients. The percentage of DRBCs was significantly higher in patients who experienced skin ulcers, priapism, or renal dysfunction. Presence of \u03b1-thalassemia deletions was associated with fewer DRBCs. A multivariable analysis model showed DRBCs to be positively associated with hemolytic parameters such as lactate dehydrogenase and bilirubin and negatively with fetal hemoglobin. The percentage of DRBCs decreased by 34% at 6 months of hydroxycarbamide (xydroxyurea) therapy. Thus, DRBCs are associated with specific clinical manifestations and biologic markers and may be a useful addition to the biologic and clinical evaluation of patients with SCD, because they can easily be measured in a hematocrit tube.<\/jats:p>","DOI":"10.1182\/blood-2012-04-424184","type":"journal-article","created":{"date-parts":[[2012,8,24]],"date-time":"2012-08-24T00:16:55Z","timestamp":1345767415000},"page":"3136-3141","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":99,"title":["Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis"],"prefix":"10.1182","volume":"120","author":[{"given":"Pablo","family":"Bartolucci","sequence":"first","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Henri-Mondor, Assistance Publique\u2013H\u00f4pitaux de Paris (AP-HP), Universit\u00e9 Paris Est, Cr\u00e9teil, France;"},{"name":"Centre de R\u00e9f\u00e9rence des syndromes dr\u00e9panocytaires majeurs, H\u00f4pital Henri-Mondor, AP-HP, Universit\u00e9 Paris Est, Cr\u00e9teil, France;"},{"name":"Inserm U955, Cr\u00e9teil, France;"}]},{"given":"Carlo","family":"Brugnara","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Children's Hospital Boston, Boston, MA;"}]},{"given":"Armando","family":"Teixeira-Pinto","sequence":"additional","affiliation":[{"name":"School of Public Health, Faculty of Medicine, University of Sydney, Sydney, Australia;"}]},{"given":"Serge","family":"Pissard","sequence":"additional","affiliation":[{"name":"Service de Biochimie, H\u00f4pital Henri-Mondor, AP-HP, Universit\u00e9 Paris Est, Cr\u00e9teil, France; and"}]},{"given":"Kamran","family":"Moradkhani","sequence":"additional","affiliation":[{"name":"Service de Biochimie, H\u00f4pital Henri-Mondor, AP-HP, Universit\u00e9 Paris Est, Cr\u00e9teil, France; and"}]},{"given":"H\u00e9l\u00e8ne","family":"Jouault","sequence":"additional","affiliation":[{"name":"Service d'H\u00e9matologie Biologique, H\u00f4pital Henri-Mondor, AP-HP, Universit\u00e9 Paris Est, Cr\u00e9teil, France"}]},{"given":"Frederic","family":"Galacteros","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Henri-Mondor, Assistance Publique\u2013H\u00f4pitaux de Paris (AP-HP), Universit\u00e9 Paris Est, Cr\u00e9teil, France;"},{"name":"Centre de R\u00e9f\u00e9rence des syndromes dr\u00e9panocytaires majeurs, H\u00f4pital Henri-Mondor, AP-HP, Universit\u00e9 Paris Est, Cr\u00e9teil, France;"},{"name":"Inserm U955, Cr\u00e9teil, France;"}]}],"member":"234","reference":[{"issue":"4748","key":"2019111810481086300_B1","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1126\/science.3961486","article-title":"Regulation of erythrocyte cation and water content in sickle cell 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