title: Alpha-thalassaemia promotes frequent vaso-occlusive crises in children with sickle cell anaemia through haemorheological changes.
abstract: BACKGROUND
  Sickle cell anaemia (SCA) is a severe hereditary haemoglobinopathy characterised by haemorheological abnormalities, which play a role in the occurrence of several acute and chronic clinical complications. While βS-haplotypes and alpha-thalassaemia modulate SCA clinical severity, their effects on blood rheology have been incompletely described. The aim of this study was to test the effects of these genetic modifiers on the haemorheological properties and clinical complication of children with SCA.
  
  
  METHODS
  Steady-state haemorheological profile, biological parameters, βS-haplotypes, alpha-globin status, vaso-occlusive crisis (VOC) and acute chest syndrome frequencies were analysed in 128 children (aged 5 to 18 years) with SCA.
  
  
  RESULTS
  Patients with alpha-thalassaemia showed increased red blood cell (RBC) deformability and aggregation compared to those without. Median VOC rate was higher in patients with homozygous alpha-thalassaemia compared to those with a normal alpha genotype. Conversely, the haemorheological profile and clinical complications were not influenced by the βS-haplotypes in our study.
  
  
  CONCLUSIONS
  Our results demonstrate that alpha-thalassaemia is associated with higher risk for VOC events in children with SCA, which may be due in part to its effects on RBC deformability and aggregation.
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.|UF Pathologie moléculaire du globule rouge, Hôpital Edouard Herriot, Lyon, France.
authors.forename: Céline
authors.initials: C
authors.lastname: Renoux
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.|Institut Universitaire de France, Paris, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Connes
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.
authors.forename: Elie
authors.initials: E
authors.lastname: Nader
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.
authors.forename: Sarah
authors.initials: S
authors.lastname: Skinner
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.
authors.forename: Camille
authors.initials: C
authors.lastname: Faes
authors.affiliation: Unité Transversale de la Drépanocytose, Centre Hospitalier Universitaire, Pointe-à-Pitre, Guadeloupe, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Petras
authors.affiliation: Institut d'hématologie et d'oncologie pédiatrique (IHOP), Hospices Civils de Lyon, Lyon, France.
authors.forename: Yves
authors.initials: Y
authors.lastname: Bertrand
authors.affiliation: Institut d'hématologie et d'oncologie pédiatrique (IHOP), Hospices Civils de Lyon, Lyon, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Garnier
authors.affiliation: Institut d'hématologie et d'oncologie pédiatrique (IHOP), Hospices Civils de Lyon, Lyon, France.
authors.forename: Daniela
authors.initials: D
authors.lastname: Cuzzubbo
authors.affiliation: Unité Transversale de la Drépanocytose, Centre Hospitalier Universitaire, Pointe-à-Pitre, Guadeloupe, France.
authors.forename: Lydia
authors.initials: L
authors.lastname: Divialle-Doumdo
authors.affiliation: Institut d'hématologie et d'oncologie pédiatrique (IHOP), Hospices Civils de Lyon, Lyon, France.
authors.forename: Kamila
authors.initials: K
authors.lastname: Kebaïli
authors.affiliation: Institut d'hématologie et d'oncologie pédiatrique (IHOP), Hospices Civils de Lyon, Lyon, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Renard
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.|Institut d'hématologie et d'oncologie pédiatrique (IHOP), Hospices Civils de Lyon, Lyon, France.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Gauthier
authors.affiliation: Unité Transversale de la Drépanocytose, Centre Hospitalier Universitaire, Pointe-à-Pitre, Guadeloupe, France.
authors.forename: Maryse
authors.initials: M
authors.lastname: Etienne-Julan
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.|Clinique de Médecine Ambulatoire/Hématologie Hôpital Edouard Herriot, Lyon, France.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Cannas
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.
authors.forename: Cyril
authors.initials: C
authors.lastname: Martin
authors.affiliation: Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.|UMR Inserm 1134, Hôpital Ricou, Centre Hospitalier Universitaire, Pointe-à-Pitre, Guadeloupe, France.
authors.forename: Marie-Dominique
authors.initials: MD
authors.lastname: Hardy-Dessources
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.|Institut Universitaire de France, Paris, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Pialoux
authors.affiliation: Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.|UMR Inserm 1134, Hôpital Ricou, Centre Hospitalier Universitaire, Pointe-à-Pitre, Guadeloupe, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Romana
authors.affiliation: Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Equipe "Biologie vasculaire et du globule rouge", Université Claude Bernard Lyon 1, COMUE, Lyon, France.|Laboratoire d'Excellence sur le globule rouge (Labex GR-Ex), Paris, France.|UF Pathologie moléculaire du globule rouge, Hôpital Edouard Herriot, Lyon, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Joly
mesh_terms: D056586:Acute Chest Syndrome / Q000473:pathology; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D004912:Erythrocytes / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D012212:Rheology; D017085:alpha-Thalassemia / Q000150:complications*
keywords: alpha-thalassaemia; haemorheology; sickle cell disease; vaso-occlusive crisis; βS-haplotypes