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

Bader‐Meunier, Brigitte; Miélot, F; Tchernia, Gil; Buisine, J.; Delsol, Georges; Duchayne, Éliane; Lemerle, S.; Leverger, Guy; Lumley, L. De; Manel, Anne‐Marie; Nathanson, M; Plantaz, Dominique; Robert, Alain; Schaison, G; Santini, Daniele; Vilmer, E

doi:10.1046/j.1365-2141.0000.d01-1480.x

Cited by 80 publications

(61 citation statements)

References 22 publications

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“…Constitutional abnormalities are often observed in children with MDS and JMML. [1][2][3][4][5][6] The unique features of Down syndrome is not appropriately addressed in the WHO classification. There are no data to indicate whether the blast threshold of 20% is better than the traditional 30% to distinguish MDS from AML in children.…”

Section: Fab and Who Classificationsmentioning

confidence: 99%

“…The WHO classifications recognizes JMML as a separate entity. There are many differences between MDS in children and adults, eg RARS is exceedingly rare in children, [1][2][3][4][5][6] deletion 5q has occasionally been observed in children [12][13][14] but the 5q− syndrome characterized by 5q− as the sole abnormality, BM blasts Ͻ5%, normal or elevated platelet count, an indolent course and long survival has not been described in children. Constitutional abnormalities are often observed in children with MDS and JMML.…”

Section: Fab and Who Classificationsmentioning

confidence: 99%

“…Myelodysplastic syndrome (MDS) in children and adolescents has hitherto received sparse attention in the pediatric literature and, despite recent attempts, [1][2][3][4][5][6] there has been no generally agreed system of classification or diagnosis. Recommendations for the classification of childhood cancer do not even mention MDS.…”

Section: Introductionmentioning

confidence: 99%

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A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases

Hasle

Niemeyer

Chessells³

et al. 2003

Leukemia

393

300

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Myelodysplastic and myeloproliferative disorders are rare in childhood and there is no widely accepted system for their diagnosis and classification. We propose minimal diagnostic criteria and a simple classification scheme which, while based on accepted morphological features and conforming with the recent suggestions of the WHO, allows for the special problems of myelodysplastic diseases in children. The classification recognizes three major diagnostic groups: (1) juvenile myelomonocytic leukemia (JMML), previously named chronic myelomonocytic leukemia (CMML) or juvenile chronic myeloid leukemia (JCML); (2) myeloid leukemia of Down syndrome, a disease with distinct clinical and biological features, encompassing both MDS and AML occurring in Down syndrome; and (3) MDS occurring both de novo and as a complication of previous therapy or pre-existing bone marrow disorder (secondary MDS). The main subtypes of MDS are refractory cytopenia (RC) and refractory anemia with excess of blasts (RAEB). It is suggested retaining the subtype of RAEB-T with 20-30% blasts in the marrow until more data are available. Cytogenetics and serial assessments of the patients are essential adjuncts to morphology both in diagnosis and classification.

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Section: Fab and Who Classificationsmentioning

confidence: 99%

Section: Fab and Who Classificationsmentioning

confidence: 99%

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A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases

Hasle

Niemeyer

Chessells³

et al. 2003

Leukemia

393

300

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“…MDS occurs predominantly in the elderly. Although several series of MDS in childhood have been reported, [1][2][3][4][5][6][7][8] epidemiological data are very limited, 1,4,8 in part because pediatric MDS is relatively rare, and also because of difficulties in diagnosis and classification. The classification of MDS is based on the morphology of peripheral blood and bone marrow proposed by the French-American-British (FAB) group in 1982.…”

Section: Introductionmentioning

confidence: 99%

Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan

et al. 2001

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We report a retrospective analysis of children with myelodysplastic syndrome (MDS) diagnosed between 1990 and 1997 in Japan. In total, 189 patients were enrolled: 122 cases of primary MDS (26 RA, 18 RAEB, 25 RAEBt, 53 CMML/JMML), 24 cases with constitutional predisposition to MDS, and 43 cases of therapy-related MDS (t-MDS). The frequency of pediatric MDS was estimated to be 7.7% of all leukemias. Cytogenetic abnormalities were observed in 41% of primary MDS and 90% of t-MDS cases. The 4-year survival rate, estimated by Kaplan-Meier analysis, for primary RA was 78.9%, while other types of MDS and JMML had rates lower than 40%, and t-MDS showed an even more unfavorable prognosis. In primary MDS, the survival rate of patients with cytogenetic abnormalities was significantly lower. Among prognostic variables by IPSS, only the cytogenetic pattern was useful for predicting outcome in childhood MDS. There was no apparent advantage to chemotherapy for RA, and the survival rate in patients with primary RA, JMML, or t-MDS receiving stem cell transplantation was significantly higher. More precise designs of our diagnostic and classification systems, as well as therapeutic trials in large-scale prospective studies, are necessary for further improvements in MDS outcome. Leukemia (2001) 15, 1713-1720.

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“…Less commonly, primary MDS occurs in the pediatric population and includes specific pediatric syndromes such as juvenile chronic myeloid leukemia and infantile monosomy 7 syndrome. [8][9][10][11][12][13][14][15] Adults under 50 years old have rarely been reported to have primary MDS. The clinical and pathologic aspects of primary MDS in younger adults and their response to bone marrow transplantation have not been widely reported.…”

Section: Introductionmentioning

confidence: 99%

Primary myelodysplasia occurring in adults under 50 years old: a clinicopathologic study of 52 patients

et al. 2002

Leukemia

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Although myelodysplastic syndromes (MDSs) are generally thought to be diseases of elderly patients, younger patients also have rarely been diagnosed with MDS. This is a report of the clinical, morphologic and cytogenetic features of 52 cases of primary MDS occurring in adults under the age of 50 years. Cases secondary to chemotherapy or radiotherapy were excluded. There were 31 males and 21 females. The median age at presentation was 39 years (range, 18 to 49 years). The interval between onset of symptoms and diagnosis was brief (median, 4 weeks; range, 1-32 weeks). Of the 49 patients for whom information about duration of symptoms was available, 13 (27%) were asymptomatic. Forty-two (81%) of the patients were classified using FAB criteria for blood and bone marrow morphology: refractory anemia (RA), 11; refractory anemia with ringed sideroblasts (RARS), four; refractory anemia with excess blasts (RAEB), 12; chronic myelomonocytic leukemia (CMML), three; refractory anemia with excess blasts in transformation (RAEB-T), 12 patients. Ten patients could not be categorized. Abnormalities involving chromosome 7 was the most frequent cytogenetic abnormality (31%). Partial chromosomal deletion and chromosome gain were also common abnormalities (22% and 9%, respectively). Translocations accounted for only 9% of the main cytogenetic abnormalities encountered in this patient population. For the 49 patients for whom information regarding AML transformation was available, 23 (47%) progressed to acute myeloid leukemia, with an overall median time to progression of 2 months (range 3 weeks to 3 years). In each category except for RARS, approximately half of the patients progressed, with a slightly less median time to progression in RAEB-T than for the other subtypes of MDS. Thirteen patients underwent bone marrow transplantation at the time of presentation of their disease. Leukemia (2002) 16, 623-631.

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

Cited by 80 publications

References 22 publications

A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases

A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases

Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan

Primary myelodysplasia occurring in adults under 50 years old: a clinicopathologic study of 52 patients

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