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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 1502–1513 (2015)Cite this article

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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.

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Author notes

  1. P L Greenberg and M Cazzola: These authors contributed equally to this work.

Authors and 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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  1. M G Della Porta
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  2. H Tuechler
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Correspondence to P L Greenberg or M Cazzola.

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Della Porta, M., Tuechler, H., Malcovati, L. et al. 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 29, 1502–1513 (2015). https://doi.org/10.1038/leu.2015.55

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