TY - JOUR
T1 - Effect of age on blood rheology in sickle cell anaemia and sickle cell haemoglobin C disease
T2 - A cross-sectional study
AU - Renoux, Céline
AU - Romana, Marc
AU - Joly, Philippe
AU - Ferdinand, Séverine
AU - Faes, Camille
AU - Lemonne, Nathalie
AU - Skinner, Sarah
AU - Garnier, Nathalie
AU - Etienne-Julan, Maryse
AU - Bertrand, Yves
AU - Petras, Marie
AU - Cannas, Giovanna
AU - Divialle-Doumdo, Lydia
AU - Nader, Elie
AU - Cuzzubbo, Daniela
AU - Lamarre, Yann
AU - Gauthier, Alexandra
AU - Waltz, Xavier
AU - Kebaili, Kamila
AU - Martin, Cyril
AU - Hot, Arnaud
AU - Hardy-Dessources, Marie Dominique
AU - Pialoux, Vincent
AU - Connes, Philippe
N1 - Publisher Copyright:
© 2016 Renoux et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
PY - 2016/6
Y1 - 2016/6
N2 - Objectives: Blood rheology plays a key role in the pathophysiology of sickle cell anaemia (SS) and sickle cell haemoglobin C disease (SC), but its evolution over the lifespan is unknown. Materials and Methods: Blood viscosity, red blood cell (RBC) deformability and aggregation, foetal haemoglobin (HbF) and haematocrit were measured in 114 healthy individuals (AA), 267 SS (161 children + 106 adults) and 138 SC (74 children + 64 adults) patients. Results: Our results showed that 1) RBC deformability is at its maximal value during the early years of life in SS and SC populations, mainly because HbF level is also at its peak, 2) during childhood and adulthood, hydroxycarbamide treatment, HbF level and gender modulated RBC deformability in SS patients, independently of age, 3) blood viscosity is higher in older SS and SC patients compared to younger ones and 4) haematocrit decreases as SS patients age. Conclusion: The hemorheological changes detected in older patients could play a role in the progressive development of several chronic disorders in sickle cell disease, whose prevalence increases with age. Retarding these age-related haemorheological impairments, by using suitable drugs, may minimize the risks of vaso-occlusive events and chronic disorders.
AB - Objectives: Blood rheology plays a key role in the pathophysiology of sickle cell anaemia (SS) and sickle cell haemoglobin C disease (SC), but its evolution over the lifespan is unknown. Materials and Methods: Blood viscosity, red blood cell (RBC) deformability and aggregation, foetal haemoglobin (HbF) and haematocrit were measured in 114 healthy individuals (AA), 267 SS (161 children + 106 adults) and 138 SC (74 children + 64 adults) patients. Results: Our results showed that 1) RBC deformability is at its maximal value during the early years of life in SS and SC populations, mainly because HbF level is also at its peak, 2) during childhood and adulthood, hydroxycarbamide treatment, HbF level and gender modulated RBC deformability in SS patients, independently of age, 3) blood viscosity is higher in older SS and SC patients compared to younger ones and 4) haematocrit decreases as SS patients age. Conclusion: The hemorheological changes detected in older patients could play a role in the progressive development of several chronic disorders in sickle cell disease, whose prevalence increases with age. Retarding these age-related haemorheological impairments, by using suitable drugs, may minimize the risks of vaso-occlusive events and chronic disorders.
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U2 - 10.1371/journal.pone.0158182
DO - 10.1371/journal.pone.0158182
M3 - Article
C2 - 27355589
AN - SCOPUS:84977624572
SN - 1932-6203
VL - 11
JO - PloS one
JF - PloS one
IS - 6
M1 - e0158182
ER -