The International Prognostic Scoring System (IPSS) for childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukemia (JMML)

H. Hasle, I. Baumann, E. Bergsträsser, S. Fenu, A. Fischer, G. Kardos, G. Kerndrup, F. Locatelli, T. Rogge, K. R. Schultz, J. Starý, M. Trebo, M. M. van den Heuvel-Eibrink, J. Harbott, P. Nöllke, C. M. Niemeyer

Research output: Contribution to journalArticlepeer-review

Abstract

The International Prognostic Scoring System (IPSS) for myelodysplastic syndrome (MDS) is based upon weighted data on bone marrow (BM) blast percentage, cytopenia, and cytogenetics, separating patients into four prognostic groups. We analyzed the value of the IPSS in 142 children with de novo MDS and 166 children with juvenile myelomonocytic leukemia (JMML) enrolled in retro- and prospective studies of the European Working Group on childhood MDS (EWOG-MDS). Survivals in MDS and JMML were analyzed separately. Among the criteria considered by the IPSS score, only BM blasts 100 × 109/l were significantly associated with a superior survival in MDS. In JMML, better survival was associated with platelets > 40 × 109/l, but not with any other IPSS factors including cytogenetics. In conclusion, the IPSS is of limited value in both pediatric MDS and JMML. The results reflect the differences between myelodysplastic and myeloproliferative diseases in children and adults.

Original languageEnglish
Pages (from-to)2008-2014
Number of pages7
JournalLeukemia
Volume18
Issue number12
DOIs
Publication statusPublished - Dec 2004

Keywords

  • Children
  • IPSS
  • JMML
  • MDS
  • Prognosis

ASJC Scopus subject areas

  • Hematology
  • Cancer Research

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