Effect of age on blood rheology in sickle cell anaemia and sickle cell haemoglobin C disease: A cross-sectional study

Céline Renoux, Marc Romana, Philippe Joly, Séverine Ferdinand, Camille Faes, Nathalie Lemonne, Sarah Skinner, Nathalie Garnier, Maryse Etienne-Julan, Yves Bertrand, Marie Petras, Giovanna Cannas, Lydia Divialle-Doumdo, Elie Nader, Daniela Cuzzubbo, Yann Lamarre, Alexandra Gauthier, Xavier Waltz, Kamila Kebaili, Cyril MartinArnaud Hot, Marie Dominique Hardy-Dessources, Vincent Pialoux, Philippe Connes

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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.

Original languageEnglish (US)
Article numbere0158182
JournalPloS one
Issue number6
StatePublished - Jun 2016

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© 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.


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