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Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis.

Abstract : Dense, dehydrated red blood cells (DRBCs) are a characteristic feature of sickle-cell disease (SCD). DRBCs play a role in the pathophysiology of SCD acute and chronic organ damage because of heightened tendency to undergo polymerization and sickling because of their higher hemoglobin S concentration. 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 α-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.
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Submitted on : Friday, February 22, 2013 - 1:46:08 PM
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Pablo Bartolucci, Carlo Brugnara, Armando Teixeira-Pinto, Serge Pissard, Kamran Moradkhani, et al.. Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis.. Blood, American Society of Hematology, 2012, 120 (15), pp.3136-41. ⟨10.1182/blood-2012-04-424184⟩. ⟨inserm-00727759⟩



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