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International Journal of Hematology

, Volume 100, Issue 5, pp 478–484 | Cite as

Cytogenetics and clinical features of pediatric myelodysplastic syndrome in Japan

  • Koichi Moriwaki
  • Atsushi Manabe
  • Takeshi Taketani
  • Akira Kikuchi
  • Tatsutoshi Nakahata
  • Yasuhide HayashiEmail author
Original Article
  • 519 Downloads

Abstract

We analyzed the cytogenetics and clinical features of pediatric myelodysplastic syndrome (MDS) in Japan. Data on patients (<16 years) diagnosed with MDS from 1990 to 2000 were retrospectively collected from pediatric hematologists in 234 institutions. Chromosome analysis was successfully performed in 255 of 277 MDS patients. The numbers of patients with refractory anemia, refractory anemia with ringed sideroblasts, refractory anemia with excess of blasts (RAEB), refractory anemia with excess of blasts in transformation (RAEBt), chronic myelomonocytic leukemia, and juvenile myelomonocytic leukemia were 67 (24 %), 51 (18 %), 51 (18 %), 20 (7 %), and 65 (23 %), respectively. The other 23 patients (8 %) could not be classified specifically. The distribution of childhood MDS in Japan according to the French–American–British subclassification was similar to that in other countries. However, we identified a higher incidence of therapy-related cases. As for relationship between cytogenetics and prognoses, abnormal karyotypes were related to poorer prognoses than normal karyotype (P < 0.01). However, patients with trisomy 8 had prognoses comparable to those with normal karyotypes. Complex karyotypes were associated with poorer prognoses among RAEB and RAEBt patients. In conclusion, prognosis of pediatric MDS is related to cytogenetics. A more precise diagnosis and classification system is needed for childhood MDS.

Keywords

Myelodysplastic syndrome Childhood Chromosomal abnormality 

Notes

Acknowledgments

We thank all the members of the Japanese Society of Pediatric Hematology-Oncology.

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© The Japanese Society of Hematology 2014

Authors and Affiliations

  • Koichi Moriwaki
    • 1
  • Atsushi Manabe
    • 2
  • Takeshi Taketani
    • 3
  • Akira Kikuchi
    • 4
  • Tatsutoshi Nakahata
    • 5
  • Yasuhide Hayashi
    • 6
    Email author
  1. 1.Department of Pediatrics, Saitama Medical CenterSaitama Medical UniversitySaitamaJapan
  2. 2.Department of PediatricsSt. Luke’s International HospitalTokyoJapan
  3. 3.Division of Blood TransfusionShimane University HospitalIzumoJapan
  4. 4.Department of PediatricsTeikyo UniversityTokyoJapan
  5. 5.Center for iPS Cell Research and ApplicationKyoto UniversityKyotoJapan
  6. 6.Department of Hematology-OncologyGunma Children’s Medical CenterShibukawaJapan

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