Myelodysplastic syndromes in childhood: Report of 49 patients from a French multicentre study

被引:73
作者
BaderMeunier, B
Mielot, F
Tchernia, G
Buisine, J
Delsol, G
Duchayne, J
Lemerle, S
Leverger, G
deLumley, L
Manel, AM
Nathanson, M
Plantaz, D
Robert, A
Schaison, G
Sommelet, D
Vilmer, E
机构
[1] Serv. de Pediat. Gen., Hôpital de Bicêtre, 94270 Le Kremlin Bicêtre
关键词
myelodysplastic syndrome; childhood; constitutional anomalies; clonality; acute myeloblastic leukaemia;
D O I
10.1046/j.1365-2141.0000.d01-1480.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:344 / 350
页数:7
相关论文
共 31 条
  • [1] APPELBAUM FR, 1987, BLOOD, V69, P92
  • [2] AUERBACH AD, 1981, PEDIATRICS, V67, P128
  • [3] REFRACTORY-ANEMIA AND MITOCHONDRIAL CYTOPATHY IN CHILDHOOD
    BADERMEUNIER, B
    ROTIG, A
    MIELOT, F
    LAVERGNE, JM
    CROISILLE, L
    RUSTIN, P
    LANDRIEU, P
    DOMMERGUES, JP
    MUNNICH, A
    TCHERNIA, G
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 1994, 87 (02) : 381 - 385
  • [4] BENNETT JM, 1986, CLIN HAEMATOL, V15, P909
  • [5] PROPOSALS FOR THE CLASSIFICATION OF THE MYELODYSPLASTIC SYNDROMES
    BENNETT, JM
    CATOVSKY, D
    DANIEL, MT
    FLANDRIN, G
    GALTON, DAG
    GRALNICK, HR
    SULTAN, C
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 1982, 51 (02) : 189 - 199
  • [6] NITROGEN MUSTARD-INDUCED CHROMOSOME BREAKAGE - A TOOL FOR FANCONIS ANEMIA DIAGNOSIS
    BERGER, R
    BERNHEIM, A
    LECONIAT, M
    VECCHIONE, D
    SCHAISON, G
    [J]. CANCER GENETICS AND CYTOGENETICS, 1980, 2 (03) : 269 - 274
  • [7] BRANDWEIN JM, 1990, AM J PEDIAT HEMATOL, V12, P63
  • [8] BROUN ER, 1990, CANCER GENET CYTOGEN, V46, P125
  • [9] CREUTZIG U, 1987, AM J PEDIAT HEMATOL, V9, P327
  • [10] EMMERSON AJB, 1992, J ROY SOC MED, V85, P495