title: [Myelodysplasia in children and mitochondrial cytopathies].
abstract: Sideroblastic anemia associated with vacuolization of haemopoietic precursors can be observed in some constitutional diseases associated with mitochondrial DNA deletion. In this condition, it is the haematological expression of a multi-tissue disorder. Haemopoiesis is polyclonal, without abnormality of nuclear DNA differing from the acquired idiopathic sideroblastic anemias which arise from a clonal transformed stem cell. This model of childhood polyclonal myelodysplasia can be observed in others myelodysplasias associated with constitutional polymalformative syndromes.
authors.affiliation: Laboratoire d'Hématologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
authors.forename: F
authors.initials: F
authors.lastname: Miélot
authors.forename: B
authors.initials: B
authors.lastname: Bader-Meunier
authors.forename: G
authors.initials: G
authors.lastname: Tchernia
authors.forename: J P
authors.initials: JP
authors.lastname: Dommergues
mesh_terms: D000756:Anemia, Sideroblastic / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D017637:Clonal Deletion; D004272:DNA, Mitochondrial; D006801:Humans; D007223:Infant; D009190:Myelodysplastic Syndromes / Q000145:classification / Q000235:genetics / Q000503:physiopathology*
chemical_list: D004272:DNA, Mitochondrial