title: Erythroid Adhesion Molecules in Sickle Cell Anaemia Infants: Insights Into Early Pathophysiology.
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.
authors.affiliation: Reference Centre for Sickle Cell Disease, Pediatric Department, Hôpital Universitaire Necker Enfants Malades, APHP, Paris, France ; Université Paris Descartes, Paris, France ; INSERM, U1134, F-75739 Paris, France ; Université Paris Diderot, Sorbonne Paris Cité, UMR_S 1134, F-75739 Paris, France ; Institut National de la Transfusion Sanguine, F-75739 Paris, France ; Laboratoire d'Excellence GR-Ex, France.
authors.forename: Valentine
authors.initials: V
authors.lastname: Brousse
authors.affiliation: INSERM, U1134, F-75739 Paris, France ; Université Paris Diderot, Sorbonne Paris Cité, UMR_S 1134, F-75739 Paris, France ; Institut National de la Transfusion Sanguine, F-75739 Paris, France ; Laboratoire d'Excellence GR-Ex, France.
authors.forename: Yves
authors.initials: Y
authors.lastname: Colin
authors.affiliation: INSERM, U1134, F-75739 Paris, France ; Université Paris Diderot, Sorbonne Paris Cité, UMR_S 1134, F-75739 Paris, France ; Institut National de la Transfusion Sanguine, F-75739 Paris, France ; Laboratoire d'Excellence GR-Ex, France.
authors.forename: Catia
authors.initials: C
authors.lastname: Pereira
authors.affiliation: Reference Centre for Sickle Cell Disease, Pediatric Department, Centre Hospitalier Intercommunal de Créteil, Créteil, France.
authors.forename: Cecile
authors.initials: C
authors.lastname: Arnaud
authors.affiliation: INSERM, U1134, F-75739 Paris, France ; Université Paris Diderot, Sorbonne Paris Cité, UMR_S 1134, F-75739 Paris, France ; Institut National de la Transfusion Sanguine, F-75739 Paris, France ; Laboratoire d'Excellence GR-Ex, France ; Reference Centre for Sickle Cell Disease, Pediatric Department, Hôpital Louis Mourier, APHP, Colombes, France.
authors.forename: Marie Helene
authors.initials: MH
authors.lastname: Odièvre
authors.affiliation: Pediatric Department, Centre Hospitalier Intercommunal Poissy-Saint-Germain-en-Laye, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Boutemy
authors.affiliation: Reference Centre for Sickle Cell Disease, Pediatric Department, Centre Hospitalier Universitaire du Kremlin Bicêtre, APHP, Le Kremlin Bicêtre, France.
authors.forename: Corinne
authors.initials: C
authors.lastname: Guitton
authors.affiliation: Reference Centre for Sickle Cell Disease, Pediatric Department, Hôpital Universitaire Necker Enfants Malades, APHP, Paris, France ; Université Paris Descartes, Paris, France.
authors.forename: Mariane
authors.initials: M
authors.lastname: de Montalembert
authors.affiliation: INSERM, U1134, F-75739 Paris, France ; Université Paris Diderot, Sorbonne Paris Cité, UMR_S 1134, F-75739 Paris, France ; Institut National de la Transfusion Sanguine, F-75739 Paris, France ; Laboratoire d'Excellence GR-Ex, France.
authors.forename: Claudine
authors.initials: C
authors.lastname: Lapouméroulie
authors.affiliation: INSERM, U1134, F-75739 Paris, France ; Université Paris Diderot, Sorbonne Paris Cité, UMR_S 1134, F-75739 Paris, France ; Institut National de la Transfusion Sanguine, F-75739 Paris, France ; Laboratoire d'Excellence GR-Ex, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Picot
authors.affiliation: INSERM, U1134, F-75739 Paris, France ; Université Paris Diderot, Sorbonne Paris Cité, UMR_S 1134, F-75739 Paris, France ; Institut National de la Transfusion Sanguine, F-75739 Paris, France ; Laboratoire d'Excellence GR-Ex, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Le Van Kim
authors.affiliation: INSERM, U1134, F-75739 Paris, France ; Université Paris Diderot, Sorbonne Paris Cité, UMR_S 1134, F-75739 Paris, France ; Institut National de la Transfusion Sanguine, F-75739 Paris, France ; Laboratoire d'Excellence GR-Ex, France.
authors.forename: Wassim
authors.initials: W
authors.lastname: El Nemer
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood* / Q000503:physiopathology; D018968:CD58 Antigens / Q000097:blood*; D015815:Cell Adhesion Molecules / Q000097:blood*; D005260:Female; D005434:Flow Cytometry; D020869:Gene Expression Profiling; D005786:Gene Expression Regulation; D006801:Humans; D007223:Infant; D008188:Lutheran Blood-Group System / Q000097:blood*; D008297:Male; D012156:Reticulocytes / Q000378:metabolism*
chemical_list: C090228:BCAM protein, human; D018968:CD58 Antigens; D015815:Cell Adhesion Molecules; C095100:ICAM4 protein, human; D008188:Lutheran Blood-Group System
keywords: Adhesion molecules; HbF; Infants; Red blood cell; Sickle cell anaemia