title: Dyserythropoiesis associated with a fas-deficient condition in childhood.
abstract: Defective lymphocyte apoptosis caused by mutations of the Fas gene can result in an autoimmune lymphoproliferative syndrome (ALPS) in humans. We report two cases of dyserythropoiesis associated with a Fas-deficient condition in childhood. In both cases, dyserythropoiesis predominated on the more mature erythroblasts, and was associated with a lymphoproliferative syndrome as well as with haemolytic anaemia, hypergammaglobulinaemia and the expansion of an unusual population of CD4- CD8- T cells that express the alpha/beta T-cell receptor. The regression of dyserythropoiesis under steroid therapy suggested that it resulted from an autoimmune mechanism, itself secondary to the lymphocyte Fas apoptosis deficiency. Fas-defective apoptosis may be a new aetiology for childhood dyserythropoiesis.
authors.affiliation: Département de Pédiatrie, Laboratoire d'Hématologie et Laboratoire de Médecine nucléaire, Hôpital de Bicêtre, Le Kremlin Bicêtre; Laboratoire d'Immunologie Pédiatrique, INSERM U429, France. brigitte.bader-meunier@bct.ap-hop-paris.fr
authors.forename: B
authors.initials: B
authors.lastname: Bader-Meunier
authors.forename: F
authors.initials: F
authors.lastname: Rieux-Laucat
authors.forename: L
authors.initials: L
authors.lastname: Croisille
authors.forename: J
authors.initials: J
authors.lastname: Yvart
authors.forename: F
authors.initials: F
authors.lastname: Mielot
authors.forename: J P
authors.initials: JP
authors.lastname: Dommergues
authors.forename: F
authors.initials: F
authors.lastname: Ledeist
authors.forename: G
authors.initials: G
authors.lastname: Tchernia
mesh_terms: D000743:Anemia, Hemolytic / Q000235:genetics*; D017209:Apoptosis / Q000235:genetics; D002648:Child; D004920:Erythropoiesis / Q000235:genetics*; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation / Q000235:genetics*; D019014:fas Receptor / Q000235:genetics*
chemical_list: D019014:fas Receptor