title: Ineffective erythropoiesis in β -thalassemia.
abstract: In humans, β -thalassemia dyserythropoiesis is characterized by expansion of early erythroid precursors and erythroid progenitors and then ineffective erythropoiesis. This ineffective erythropoiesis is defined as a suboptimal production of mature erythrocytes originating from a proliferating pool of immature erythroblasts. It is characterized by (1) accelerated erythroid differentiation, (2) maturation blockade at the polychromatophilic stage, and (3) death of erythroid precursors. Despite extensive knowledge of molecular defects causing β -thalassemia, less is known about the mechanisms responsible for ineffective erythropoiesis. In this paper, we will focus on the underlying mechanisms leading to premature death of thalassemic erythroid precursors in the bone marrow.
authors.affiliation: Centre National de la Recherche Scientifique-Unité Mixte de Recherche 8147, Université Paris V, René Descartes, Hôpital Necker, Paris, France. jean-antoine.ribeil@nck.aphp.fr
authors.forename: Jean-Antoine
authors.initials: JA
authors.lastname: Ribeil
authors.forename: Jean-Benoit
authors.initials: JB
authors.lastname: Arlet
authors.forename: Michael
authors.initials: M
authors.lastname: Dussiot
authors.forename: Ivan Cruz
authors.initials: IC
authors.lastname: Moura
authors.forename: Geneviève
authors.initials: G
authors.lastname: Courtois
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
mesh_terms: D001854:Bone Marrow Cells / Q000473:pathology*; D002454:Cell Differentiation; D004912:Erythrocytes / Q000473:pathology*; D004920:Erythropoiesis; D006801:Humans; D008954:Models, Biological; D013234:Stem Cells / Q000473:pathology*; D017086:beta-Thalassemia / Q000473:pathology* / Q000503:physiopathology*