Biomechanics and biorheology of red blood cells in sickle cell anemia |
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| Institution: | 1. Inserm UMR 1134, Hôpital Ricou, CHU de Pointe-à-Pitre, 97157 Pointe-à-Pitre, Guadeloupe;2. Laboratory of Excellence GR-Ex «The red cell: from genesis to death», PRES Sorbonne Paris Cité, 75015 Paris, France;3. Institut Universitaire de France, Paris, France;4. Laboratoire CRIS EA647, Section “Vascular Biology and Red Blood Cell”, Université Claude Bernard Lyon 1, 69100 Villeurbanne, France;5. Division of Cardiology, Emory University School of Medicine, Atlanta, GA, USA;6. Section of Cardiology, Atlanta VA Medical Center, Decatur, GA, USA;7. Children''s Hospital Los Angeles, Division of Cardiology, USA;8. Department of Medicine, Cardeza Foundation for Hematologic Research, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA |
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| Abstract: | Sickle cell anemia (SCA) is an inherited blood disorder that causes painful crises due to vaso-occlusion of small blood vessels. The primary cause of the clinical phenotype of SCA is the intracellular polymerization of sickle hemoglobin resulting in sickling of red blood cells (RBCs) in deoxygenated conditions. In this review, we discuss the biomechanical and biorheological characteristics of sickle RBCs and sickle blood as well as their implications toward a better understanding of the pathophysiology and pathogenesis of SCA. Additionally, we highlight the adhesive heterogeneity of RBCs in SCA and their specific contribution to vaso-occlusive crisis. |
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| Keywords: | Sickle cell disease Erythrocyte Biomechanics Biorheology Adhesion |
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