[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: F Miélot, B Bader-Meunier, G Tchernia, J P Dommergues
• Journal: Pathologie-biologie (Pathol Biol (Paris)) Vol. 45 Issue 7 Pg. 594-9 (Sep 1997) ISSN: 0369-8114 [Print] France
• Vernacular Title: Myélodysplasies de l'enfant et cytopathies mitochondriales.
• PMID: 9404485 (Publication Type: English Abstract, Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• DNA, Mitochondrial

Topics

• Anemia, Sideroblastic (physiopathology)
• Child
• Child, Preschool
• Chromosome Deletion
• Clonal Deletion
• DNA, Mitochondrial
• Humans
• Infant
• Myelodysplastic Syndromes (classification, genetics, physiopathology)