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Myelodysplastic syndromes in childhood: report of 49 patients from a French multicentre study
Author(s) -
BaderMeunier Brigitte,
Miélot Françoise,
Tchernia Gil,
Buisine Jacqueline,
Delsol Georges,
Duchayne Eliane,
Lemerle Sophie,
Leverger Guy,
De Lumley Lionel,
Manel AnneMarie,
Nathanson Mireille,
Plantaz Dominique,
Robert Alain,
Schaison Gérard,
Sommelet DaniÈle,
Vilmer Etienne
Publication year - 1996
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.0000.d01-1480.x
Subject(s) - myelodysplastic syndromes , medicine , pediatrics , bone marrow , hypoplasia
We describe the clinical, cytological and cytogenetic features of 49 cases of myelodysplastic syndromes (MDS) in childhood. Three children had received prior cytotoxic treatment (group 1); all of these had cytogenetic abnormalities and died shortly after diagnosis. 22 children had constitutional anomalies (group 2). The remaining 24 MDS were considered as ‘primary’ (group 3). Hypoplastic marrow was found in nine cases, and only 53% of the MDS fitted the adult FAB classification. Transformation to AML occurred in 11 cases, development of aplastic anaemia in three cases, and spontaneous remission in one case each of RA and RAEB. Differences were observed between groups 2 and 3 in terms of mean age at diagnosis (11.1 months v 5 years), rate of cytogenetic anomalies (15% v 38%) and rate of progression towards acute leukaemia (13% v 29%). In group 2, all the four girls studied exhibited a polyclonal pattern of X‐inactivation, which suggests that MDS may be only the haematological expression of an embryological defect with different target tissues. This study suggests that some MDS in childhood can exhibit particular features such as congenital anomalies associated with MDS, bone marrow hypoplasia, polyclonality, and spontaneous remission. It emphasizes that the FAB classification is not adequate for children and addresses the question of whether these MDS are always malignant diseases.