Abstract:
:Sickle cell anaemia (SCA) results from a single mutation in the β globin gene. It is seldom symptomatic in the first semester of life. We analysed the expression pattern of 9 adhesion molecules on red blood cells, in a cohort of 54 SCA and 17 non-SCA very young infants of comparable age (median 144 days, 81-196). Haemoglobin F (HbF) level was unsurprisingly elevated in SCA infants (41.2% ± 11.2) and 2-4 fold higher than in non-SCA infants, yet SCA infants presented significantly decreased Hb level and increased reticulocytosis. Cytometry analysis evidenced a specific expression profile on reticulocytes of SCA infants, with notably an increased expression of the adhesion molecules Lu/BCAM, ICAM-4 and LFA-3, both in percentage of positive cells and in surface density. No significant difference was found on mature red cells. Our findings demonstrate the very early onset of reticulocyte membrane modifications in SCA asymptomatic infants and allow an insight into the first pathological changes with the release of stress reticulocytes expressing a distinctive profile of adhesion molecules.
journal_name
EBioMedicinejournal_title
EBioMedicineauthors
Brousse V,Colin Y,Pereira C,Arnaud C,Odièvre MH,Boutemy A,Guitton C,de Montalembert M,Lapouméroulie C,Picot J,Le Van Kim C,El Nemer Wdoi
10.1016/j.ebiom.2014.12.006subject
Has Abstractpub_date
2014-12-18 00:00:00pages
154-7issue
2issn
2352-3964pii
S2352-3964(14)00058-9journal_volume
2pub_type
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