title: [Regressive myelodysplastic syndromes in children. Committee on Childhood Myelodysplasia of the Society of Pediatric Hematology and Immunology].
abstract: BACKGROUND
  As in adults, myelodysplastic syndromes in children are often preleukemic and their spontaneous remission is exceptional.
  
  
  METHODS
  Case 1. A girl, aged 8 years, developed pancytopenia with macrocytic anemia. The myelogram and bone marrow biopsy showed dyserythropoiesis with macroblastosis, but without sideroblasts. The chromosome configuration was normal and the negative results of other investigations led to the diagnosis of refractory anemia without any known cause. Bone marrow transplantation was considered, but a spontaneous remission intervened and has persisted for 3 years. Case 2. A girl, aged 5 years, was investigated for worsening of a macrocytic anemia that had been present for 3 years, without changes of granulocytes and platelets. The myelogram showed dyserythropoiesis with megaloblastosis and sideroblasts. No known cause could be identified. As for the preceding patient, a spontaneous remission occurred, and has persisted for 3 years. Case 3. A girl, aged 5 months, developed pancytopenia. The myelogram showed dys-megakaryocytopoiesis. All hematologic data spontaneously normalized after 1 year, and have remained so 1 year later.
  
  
  CONCLUSIONS
  The spontaneous remission, that has occurred in our 3 patients, has been reported in one only adult. This possibility precludes considering all the myelodysplastic syndrome in children as preleukemic, and suggests that bone marrow transplantation is not urgent in these situations.
authors.affiliation: Service de Pédiatrie Générale, CHU Bicêtre, Le Kremlin-Bicêtre.
authors.forename: B
authors.initials: B
authors.lastname: Bader-Meunier
authors.forename: F
authors.initials: F
authors.lastname: Thollot
authors.forename: F
authors.initials: F
authors.lastname: Mielot
authors.forename: J
authors.initials: J
authors.lastname: Yvart
authors.forename: J P
authors.initials: JP
authors.lastname: Dommergues
authors.forename: D
authors.initials: D
authors.lastname: Sommelet
authors.forename: G
authors.initials: G
authors.lastname: Tchernia
mesh_terms: D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D009190:Myelodysplastic Syndromes / Q000175:diagnosis; D012075:Remission, Spontaneous
vernacular_title: Myélodysplasies régressives de l'enfant. Groupe de Travail sur les Myélodysplasies de l'Enfant de la Société d'Hématologie et d'Immunologie Pédiatrique (SHIP).