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Alpha‐thalassaemia promotes frequent vaso‐occlusive crises in children with sickle cell anaemia through haemorheological changes
Author(s) -
Renoux Céline,
Connes Philippe,
Nader Elie,
Skinner Sarah,
Faes Camille,
Petras Marie,
Bertrand Yves,
Garnier Nathalie,
Cuzzubbo Daniela,
DivialleDoumdo Lydia,
Kebaïli Kamila,
Renard Cécile,
Gauthier Alexandra,
EtienneJulan Maryse,
Cannas Giovanna,
Martin Cyril,
HardyDessources MarieDominique,
Pialoux Vincent,
Romana Marc,
Joly Philippe
Publication year - 2017
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26455
Subject(s) - medicine , hemorheology , acute chest syndrome , erythrocyte deformability , alpha thalassemia , sickle cell anemia , thalassemia , haplotype , red blood cell , blood viscosity , alpha (finance) , genotype , gastroenterology , surgery , disease , genetics , construct validity , biology , patient satisfaction , gene
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 β S ‐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. Procedure Steady‐state haemorheological profile, biological parameters, β S ‐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 β S ‐haplotypes in our study. Conclusion 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.