[Myelodysplasia in children and mitochondrial cytopathies].
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.
Miélot
F
F
Laboratoire d'Hématologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Bader-Meunier
B
B
Tchernia
G
G
Dommergues
J P
JP
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*
D004272:DNA, Mitochondrial