Original Article | Published:

Myelodysplasias

Validation of WHO classification-based Prognostic Scoring System (WPSS) for myelodysplastic syndromes and comparison with the revised International Prognostic Scoring System (IPSS-R). A study of the International Working Group for Prognosis in Myelodysplasia (IWG-PM)

Leukemia volume 29, pages 15021513 (2015) | Download Citation

Abstract

A risk-adapted treatment strategy is mandatory for myelodysplastic syndromes (MDS). We refined the World Health Organization (WHO)-classification-based Prognostic Scoring System (WPSS) by determining the impact of the newer clinical and cytogenetic features, and we compared its prognostic power to that of the revised International Prognostic Scoring System (IPSS-R). A population of 5326 untreated MDS was considered. We analyzed single WPSS parameters and confirmed that the WHO classification and severe anemia provide important prognostic information in MDS. A strong correlation was found between the WPSS including the new cytogenetic risk stratification and WPSS adopting original criteria. We then compared WPSS with the IPSS-R prognostic system. A highly significant correlation was found between the WPSS and IPSS-R risk classifications. Discrepancies did occur among lower-risk patients in whom the number of dysplastic hematopoietic lineages as assessed by morphology did not reflect the severity of peripheral blood cytopenias and/or increased marrow blast count. Moreover, severe anemia has higher prognostic weight in the WPSS versus IPSS-R model. Overall, both systems well represent the prognostic risk of MDS patients defined by WHO morphologic criteria. This study provides relevant in formation for the implementation of risk-adapted strategies in MDS.

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Acknowledgements

We thank the MDS Foundation Inc., for statistical support (HT), Ms Tracey Iraca and MDS Foundation staff for helpful logistical assistance for the IWG-PM project. The study was supported by Fondazione Berlucchi, Brescia, Fondazione Veronesi, Milan, Fondazione IRCCS Policlinico San Matteo, Pavia, and Fondazione Cariplo/Regione Lombardia, Milan, Italy (MGDP and MC), and by Associazione Italiana per la Ricerca sul Cancro, Milan, Italy (LM and MC).

Author Contributions

MGDP, HT, LM, PLG and MC designed, performed and coordinated the research, collected, contributed, analyzed and interpreted the data, and wrote the manuscript; HT designed and performed the research, performed the statistical analyses, produced the figures and edited the manuscript; JS, GS, GG-M, FS, JMB, DB, PF, FD, HK, AK, AL, JC, CF, MMLB, MLS, OK, ML, JM, SMMM, YM, MP, MS, WRS, RS, ST, PV, TV, AAvdL, UG and DH collected and contributed data and critically reviewed the manuscript.

Author information

Author notes

    • P L Greenberg
    •  & M Cazzola

    These authors contributed equally to this work.

Affiliations

  1. Department of Hematology Oncology, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy

    • M G Della Porta
    • , L Malcovati
    •  & M Cazzola
  2. Department of Internal Medicine, University of Pavia, Pavia, Italy

    • M G Della Porta
  3. Hanusch Hospital, Boltzmann Institute for Leukemia Research, Vienna, Austria

    • H Tuechler
  4. Department of Molecular Medicine, University of Pavia, Pavia, Italy

    • L Malcovati
    •  & M Cazzola
  5. Georg August Universität, Göttingen, Germany

    • J Schanz
    •  & D Haase
  6. Hospital Universitario La Fe, Valencia, Spain

    • G Sanz
  7. The University of Texas, MD Anderson Cancer Center, Houston, TX, USA

    • G Garcia-Manero
    •  & H Kantarjian
  8. Institut de Recerca contra la Leucèmia Josep Carreras, Barcelona, Spain

    • F Solé
  9. James P. Wilmot Cancer Institute, University of Rochester Medical Center, Rochester, NY, USA

    • J M Bennett
  10. St James's University Hospital, Leeds, UK

    • D Bowen
  11. Hôpital Avicenne, Assistance Publique–Hôpitaux de Paris (AP-HP)/University Paris XIII, Bobigny, France

    • P Fenaux
  12. Hôpital Cochin, AP-HP University of Paris V, Paris, France

    • F Dreyfus
  13. Heinrich-Heine University Hospital, Düsseldorf, Germany

    • A Kuendgen
    •  & U Germing
  14. Fondazione Italiana Sindromi Mielodisplastiche c/o SS Antonio e Biagio Hospital, Alessandria, Italy

    • A Levis
  15. Institute of Hematology and Blood Transfusion, Praha, Czech Republic

    • J Cermak
  16. Medical University of Vienna, Vienna, Austria

    • C Fonatsch
    • , W R Sperr
    •  & P Valent
  17. University of Chicago Comprehensive Cancer Research Center, Chicago, IL, USA

    • M M Le Beau
  18. Quest Diagnostics Nichols Institute, Chantilly, VA, USA

    • M L Slovak
  19. Elisabethinen Hospital, Linz, Austria

    • O Krieger
  20. University of Freiburg Medical Center, Freiburg, Germany

    • M Luebbert
  21. Cleveland Clinic, Cleveland, OH, USA

    • J Maciejewski
    •  & M A Sekeres
  22. Federal University of Ceara, Fortaleza, Brazil

    • S M M Magalhaes
  23. Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan

    • Y Miyazaki
  24. Hanusch Hospital and L. Boltzmann Cluster Oncology, Vienna, Austria

    • M Pfeilstöcker
    •  & R Stauder
  25. University of Dundee, Dundee, Scotland, UK

    • S Tauro
  26. Hospital Universitario Vall d'Hebron, Barcelona, Spain

    • T Vallespi
  27. Department of Hematology, VU University Medical Center, Amsterdam, The Netherlands

    • A A van de Loosdrecht
  28. Division of Hematology, Stanford University Cancer Center, Stanford, CA, USA

    • P L Greenberg

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Competing interests

The authors declare no conflict of interest.

Corresponding authors

Correspondence to P L Greenberg or M Cazzola.

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DOI

https://doi.org/10.1038/leu.2015.55

Supplementary Information accompanies this paper on the Leukemia website (http://www.nature.com/leu)

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