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Blood rheological abnormalities in sickle cell anemia.
Clin Hemorheol Microcirc. 2018; 68(2-3):165-172.CH

Abstract

This review focuses on the contribution of abnormal blood rheology in the pathophysiology of sickle cell anemia (SCA). SCA is characterized by a reduction of red blood cell (RBC) deformability but this reduction is very heterogeneous among patients. Recent works have shown that patients with the lowest RBC deformability (measured by ektacytometry) have enhanced hemolysis and would be more prone to develop several complications such as priapism, leg ulcers and glomerulopathy. In contrast, patients with the highest deformability, and not under hydroxyurea therapy, seem to develop more frequently vaso-occlusive like events. Although less studied, RBC aggregation properties are very different between SCA and healthy individuals and it was demonstrated that increased RBC aggregates strength could be involved in some complications. Finally, several studies have established that the vascular system of SCA patients could not fully compensate any increase in blood viscosity because of the loss of vascular reactivity, which may result in vaso-occlusive crises.

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Authors+Show Affiliations

Connes P
Laboratoire LIBM EA7424, Team"Vascular Biology and Red Blood Cell", Université Claude Bernard Lyon 1, University of Lyon, 69100 Villeurbanne, France. Laboratory of Excellence GR-Ex « The red cell: from genesis to death », PRES Sorbonne Paris Cité, 75015, Paris, France. Institut Universitaire de France, Paris, France.
Renoux C
Laboratoire LIBM EA7424, Team"Vascular Biology and Red Blood Cell", Université Claude Bernard Lyon 1, University of Lyon, 69100 Villeurbanne, France. Laboratory of Excellence GR-Ex « The red cell: from genesis to death », PRES Sorbonne Paris Cité, 75015, Paris, France. Laboratoire de biochimie des pathologies érythrocytaires, Centre de Biologie Est, Hospices Civils de Lyon, France.
Romana M
Laboratory of Excellence GR-Ex « The red cell: from genesis to death », PRES Sorbonne Paris Cité, 75015, Paris, France. Inserm UMR 1134, Hôpital Ricou, CHU de Pointe-à-Pitre, 97157 Pointe-à-Pitre, Guadeloupe.
Abkarian M
CNRS UMR 5048, Université de Montpellier, Centre de Biochimie Structurale, 34090 Montpellier, France.
Joly P
Laboratoire LIBM EA7424, Team"Vascular Biology and Red Blood Cell", Université Claude Bernard Lyon 1, University of Lyon, 69100 Villeurbanne, France. Laboratory of Excellence GR-Ex « The red cell: from genesis to death », PRES Sorbonne Paris Cité, 75015, Paris, France. Laboratoire de biochimie des pathologies érythrocytaires, Centre de Biologie Est, Hospices Civils de Lyon, France.
Martin C
Laboratoire LIBM EA7424, Team"Vascular Biology and Red Blood Cell", Université Claude Bernard Lyon 1, University of Lyon, 69100 Villeurbanne, France. Laboratory of Excellence GR-Ex « The red cell: from genesis to death », PRES Sorbonne Paris Cité, 75015, Paris, France.
Hardy-Dessources MD
Laboratory of Excellence GR-Ex « The red cell: from genesis to death », PRES Sorbonne Paris Cité, 75015, Paris, France. Inserm UMR 1134, Hôpital Ricou, CHU de Pointe-à-Pitre, 97157 Pointe-à-Pitre, Guadeloupe.
Ballas SK
Department of Medicine, Cardeza Foundation for Hematologic Research, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA.

MeSH

Anemia, Sickle CellErythrocyte DeformabilityFemaleHumansMaleRheology

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29614630

Citation

Connes, Philippe, et al. "Blood Rheological Abnormalities in Sickle Cell Anemia." Clinical Hemorheology and Microcirculation, vol. 68, no. 2-3, 2018, pp. 165-172.
Connes P, Renoux C, Romana M, et al. Blood rheological abnormalities in sickle cell anemia. Clin Hemorheol Microcirc. 2018;68(2-3):165-172.
Connes, P., Renoux, C., Romana, M., Abkarian, M., Joly, P., Martin, C., Hardy-Dessources, M. D., & Ballas, S. K. (2018). Blood rheological abnormalities in sickle cell anemia. Clinical Hemorheology and Microcirculation, 68(2-3), 165-172. https://doi.org/10.3233/CH-189005
Connes P, et al. Blood Rheological Abnormalities in Sickle Cell Anemia. Clin Hemorheol Microcirc. 2018;68(2-3):165-172. PubMed PMID: 29614630.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Blood rheological abnormalities in sickle cell anemia. AU - Connes,Philippe, AU - Renoux,Céline, AU - Romana,Marc, AU - Abkarian,Manouk, AU - Joly,Philippe, AU - Martin,Cyril, AU - Hardy-Dessources,Marie-Dominique, AU - Ballas,Samir K, PY - 2018/4/5/entrez PY - 2018/4/5/pubmed PY - 2018/5/31/medline KW - Sickle cell anemia KW - blood viscosity KW - red blood cell aggregation KW - red blood cell deformability SP - 165 EP - 172 JF - Clinical hemorheology and microcirculation JO - Clin Hemorheol Microcirc VL - 68 IS - 2-3 N2 - This review focuses on the contribution of abnormal blood rheology in the pathophysiology of sickle cell anemia (SCA). SCA is characterized by a reduction of red blood cell (RBC) deformability but this reduction is very heterogeneous among patients. Recent works have shown that patients with the lowest RBC deformability (measured by ektacytometry) have enhanced hemolysis and would be more prone to develop several complications such as priapism, leg ulcers and glomerulopathy. In contrast, patients with the highest deformability, and not under hydroxyurea therapy, seem to develop more frequently vaso-occlusive like events. Although less studied, RBC aggregation properties are very different between SCA and healthy individuals and it was demonstrated that increased RBC aggregates strength could be involved in some complications. Finally, several studies have established that the vascular system of SCA patients could not fully compensate any increase in blood viscosity because of the loss of vascular reactivity, which may result in vaso-occlusive crises. SN - 1875-8622 UR - https://www.unboundmedicine.com/medline/citation/29614630/Blood_rheological_abnormalities_in_sickle_cell_anemia_ DB - PRIME DP - Unbound Medicine ER -