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MDS and JMML

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

Leukemia volume 18, pages 2008–2014 (2004)Cite this article

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 <5% and platelets >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.

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References

  1. Passmore SJ, Hann IM, Stiller CA, Ramani P, Swansbury GJ, Gibbons B et al. Pediatric myelodysplasia: a study of 68 children and a new prognostic scoring system. Blood 1995; 85: 1742–1750.

    CAS  PubMed  Google Scholar 

  2. Hasle H, Kerndrup G, Jacobsen BB . Childhood myelodysplastic syndrome in Denmark: incidence and predisposing conditions. Leukemia 1995; 9: 1569–1572.

    CAS  PubMed  Google Scholar 

  3. Bader-Meunier B, Mielot F, Tchernia G, Buisine J, Delsol G, Duchayne E et al. Myelodysplastic syndrome in childhood: report of 49 patients from a French multicenter study. Br J Haematol 1996; 92: 344–350.

    Article  CAS  PubMed  Google Scholar 

  4. Luna-Fineman S, Shannon KM, Atwater SK, Davis J, Masterson M, Ortega J et al. Myelodysplastic and myeloproliferative disorders of childhood: a study of 167 patients. Blood 1999; 93: 459–466.

    CAS  PubMed  Google Scholar 

  5. Hasle H, Wadsworth LD, Massing BG, McBride M, Schultz KR . A population-based study of childhood myelodysplastic syndrome in British Columbia, Canada. Br J Haematol 1999; 106: 1027–1032.

    Article  CAS  PubMed  Google Scholar 

  6. Sasaki H, Manabe A, Kojima S, Tsuchida M, Hayashi Y, Ikuta K et al. Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan. Leukemia 2001; 15: 1713–1720.

    Article  CAS  PubMed  Google Scholar 

  7. Passmore SJ, Chessells JM, Kempski H, Hann IM, Brownbill PA, Stiller CA . Paediatric MDS and JMML in the UK: a population based study of incidence and survival. Br J Haematol 2003; 121: 758–767.

    Article  PubMed  Google Scholar 

  8. Anderson JE, Appelbaum FR, Schoch G, Gooley T, Anasetti C, Bensinger WI et al. Allogeneic marrow transplantation for refractory anemia: a comparison of two preparative regimens and analysis of prognostic factors. Blood 1996; 87: 51–58.

    CAS  PubMed  Google Scholar 

  9. Kardos G, Baumann I, Passmore SJ, Locatelli F, Hasle H, Schultz KR et al. Refractory anemia in childhood: a retrospective analysis of 67 patients with particular reference to monosomy 7. Blood 2003; 102: 1997–2003.

    Article  CAS  PubMed  Google Scholar 

  10. Castro-Malaspina H, Schaison G, Passe S, Pasquier A, Berger R, Bayle-Weisgerber C et al. Subacute and chronic myelomonocytic leukemia in children (juvenile CML). Clinical and hematologic observations, and identification of prognostic factors. Cancer 1984; 54: 675–686.

    Article  CAS  PubMed  Google Scholar 

  11. Owen G, Lewis IJ, Morgan M, Robinson A, Stevens RF . Prognostic factors in juvenile chronic granulocytic leukaemia. Br J Cancer Suppl 1992; 18: S68–S71.

    CAS  PubMed  PubMed Central  Google Scholar 

  12. Niemeyer CM, Aricò M, Basso G, Cantù-Rajnoldi A, Creutzig U, Haas OA, et al., Members of the European Working Group on Myelodysplastic Syndromes in Childhood (EWOG-MDS). Chronic myelomonocytic leukemia in childhood: a retrospective analysis of 110 cases. Blood 1997; 89: 3534–3543.

    CAS  PubMed  Google Scholar 

  13. Greenberg P, Cox C, Le Beau MM, Fenaux P, Morel P, Sanz G et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997; 89: 2079–2088.

    CAS  PubMed  Google Scholar 

  14. Hasle H, Niemeyer CM, Chessells JM, Baumann I, Bennett JM, Kerndrup G et al. A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases. Leukemia 2003; 17: 277–282.

    Article  CAS  PubMed  Google Scholar 

  15. Hasle H, Aricò M, Basso G, Biondi A, Cantù-Rajnoldi A, Creutzig U et al. Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7. Leukemia 1999; 13: 376–385.

    Article  CAS  PubMed  Google Scholar 

  16. Kaplan EL, Meier P . Nonparametric estimation from incomplete observations. J Am Stat Assoc 1958; 53: 457–481.

    Article  Google Scholar 

  17. Cox DR . Regression models and life tables. J R Stat Soc B 1972; 34: 187–220.

    Google Scholar 

  18. Hosmer DW, Lemeshow S . Applied Survival Analysis. Regression Modeling of Time to Event Data. New York: Wiley, 1999.

    Google Scholar 

  19. Chang KL, O'Donnell MR, Slovak ML, Dagis AC, Arber DA, Niland JC et al. Primary myelodysplasia occurring in adults under 50 years old: a clinicopathologic study of 52 patients. Leukemia 2002; 16: 623–631.

    Article  CAS  PubMed  Google Scholar 

  20. Woods WG, Barnard DR, Alonzo TA, Buckley JD, Kobrinsky N, Arthur DC et al. Prospective study of 90 children requiring treatment for juvenile myelomonocytic leukemia or myelodysplastic syndrome: a report from the Children's Cancer Group. J Clin Oncol 2002; 20: 434–440.

    PubMed  Google Scholar 

  21. Nevill TJ, Fung HC, Shepherd JD, Horsman DE, Nantel SH, Klingemann HG et al. Cytogenetic abnormalities in primary myelodysplastic syndrome are highly predictive of outcome after allogeneic bone marrow transplantation. Blood 1998; 92: 1910–1917.

    CAS  PubMed  Google Scholar 

  22. Manabe A, Okamura J, Yumura-Yagi K, Akiyama Y, Sako M, Uchiyama H et al. Allogeneic hematopoietic stem cell transplantation for 27 children with juvenile myelomonocytic leukemia diagnosed based on the criteria of the International JMML Working Group. Leukemia 2002; 16: 645–649.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

This work was supported in part by grants from the Danish Childhood Cancer Foundation (Børnecancerfonden), the German José Carreras Leukemia Foundation, and the German BMBF Competence Network Pediatric Oncology (Project E: Preleukemic Bone Marrow Disorders).

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Authors and Affiliations

  1. Department of Pediatrics, Skejby Hospital, Aarhus University, Denmark

    H Hasle

  2. Institute of Pathology, Erlangen, Germany

    I Baumann

  3. Department of Pediatrics, Zurich, Switzerland

    E Bergsträsser

  4. Dipartimento di Ematologia, Università La Sapienza, Roma, Italy

    S Fenu

  5. Department of Pediatrics and Adolescent Medicine, University of Freiburg, Germany

    A Fischer, T Rogge, P Nöllke & C M Niemeyer

  6. VU university Medical Center, Amsterdam, The Netherlands

    G Kardos

  7. Department of Pathology, Odense University Hospital, Odense, Denmark

    G Kerndrup

  8. Oncoematologia Pediatrica, IRCCS Policlinico San Matteo, Pavia, Italy

    F Locatelli

  9. Department of Pediatrics, University of British Columbia, Vancouver, Canada

    K R Schultz

  10. Department of Pediatrics, University Hospital Motol, Prague, Czech Republic

    J Starý

  11. St Anna Kinderspital, Vienna, Austria

    M Trebo

  12. Department of Pediatric Oncology/Hematology, Sophia Children's Hospital, Erasmus University Medical Center, Rotterdam, The Netherlands

    M M van den Heuvel-Eibrink

  13. Department of Pediatric Hematology and Oncology, University of Giessen, Germany

    J Harbott

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  1. H Hasle
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  7. G Kerndrup
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  16. C M Niemeyer
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for the European Working Group on childhood MDS (EWOG-MDS)

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Correspondence to H Hasle.

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Hasle, H., Baumann, I., Bergsträsser, E. et al. The International Prognostic Scoring System (IPSS) for childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukemia (JMML). Leukemia 18, 2008–2014 (2004). https://doi.org/10.1038/sj.leu.2403489

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