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Journal Article Epublish 40 citations

PubMed: 27355589 DOI: 10.1371/journal.pone.0158182

Effect of Age on Blood Rheology in Sickle Cell Anaemia and Sickle Cell Haemoglobin C Disease: A Cross-Sectional Study.

PLoS One 11(6):e0158182

Authors: 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 Martin, Arnaud Hot, Marie-Dominique Hardy-Dessources, Vincent Pialoux, Philippe Connes

Affiliations

  • Unité de Pathologie Moléculaire du Globule Rouge, Laboratoire de Biochimie et de Biologie Moléculaire, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
  • Laboratoire Interuniversitaire de Biologie de la Motricité, Equipe "Vascular Biology and Red Blood Cell", Université Claude Bernard Lyon 1, Lyon, France.
  • Laboratoire d'Excellence du Globule Rouge (LABEX GR-Ex), PRES Sorbonne, Paris, France.
  • UMR Inserm U1134, Université des Antilles et de la Guyane, Hôpital de Pointe-à-Pitre, Hôpital Ricou, 97159 Pointe-à-Pitre Cedex, Guadeloupe, France.
  • Unité Transversale de la Drépanocytose, Hôpital de Pointe-à-Pitre, Hôpital Ricou, 97159 Pointe-à-Pitre Cedex, Guadeloupe, France.
  • Institut d'Hématologie et d'Oncologie Pédiatrique, Hospices Civils de Lyon, Lyon, France.
  • Service de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
  • Institut Universitaire de France (IUF), Paris, France.

Abstract

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.

MeSH

Cells, Cross-Sectional Studies, Blood, Rheology, Anemia, Child

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