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Myelodysplasia

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

Leukemia volume 15, pages 1713–1720 (2001)Cite this article

Abstract

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.

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Acknowledgements

The authors express sincere gratitude to the members of Japan Pediatric Hematology Society for their cooperation in this study. We also thank Dr Ichiro Tsukimoto for providing the data of registry on childhood leukemia and aplastic anemia.

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Authors and Affiliations

  1. Department of Pediatrics, Kawasaki Kyodo Hospital, Japan

    H Sasaki

  2. Institute of Medical Science, University of Tokyo, Japan

    A Manabe

  3. Department of Pediatrics, Nagoya University, School of Medicine, Japan

    S Kojima

  4. Ibaraki Children's Hospital, Japan

    M Tsuchida

  5. Department of Pediatrics, University of Tokyo, School of Medicine, Japan

    Y Hayashi

  6. Department of Blood Transfusion, Yokohama City University, School of Medicine, Japan

    K Ikuta

  7. Institute for Clinical Research, National Kyushu Cancer Center, Japan

    J Okamura

  8. Department of Pediatrics, Shinshu University, School of Medicine, Japan

    K Koike

  9. First Department of Pediatrics, Toho University, School of Medicine, Japan

    A Ohara

  10. Division of Pediatrics, Hamanomachi Hospital, Japan

    E Ishii

  11. Department of Pediatrics, Mie University, School of Medicine, Japan

    Y Komada

  12. Department of Pediatrics, Kyoto Prefectual University, School of Medicine, Japan

    S Hibi

  13. Department of Pediatrics, Kyoto University, School of Medicine, Japan

    T Nakahata

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Sasaki, H., Manabe, A., Kojima, S. et al. Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan. Leukemia 15, 1713–1720 (2001). https://doi.org/10.1038/sj.leu.2402271

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  • DOI: https://doi.org/10.1038/sj.leu.2402271

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