Blood Journal
Leading the way in experimental and clinical research in hematology

Revised International Prognostic Scoring System (IPSS-R) for myelodysplastic syndromes

  1. Peter L. Greenberg1,*,
  2. Heinz Tuechler2,
  3. Julie Schanz3,
  4. Guillermo Sanz4,
  5. Guillermo Garcia-Manero5,
  6. Francesc Solé6,
  7. John M. Bennett7,
  8. David Bowen8,
  9. Pierre Fenaux9,
  10. Francois Dreyfus10,
  11. Hagop Kantarjian5,
  12. Andrea Kuendgen11,
  13. Alessandro Levis12,
  14. Luca Malcovati13,
  15. Mario Cazzola13,
  16. Jaroslav Cermak14,
  17. Christa Fonatsch15,
  18. Michelle M. Le Beau16,
  19. Marilyn L. Slovak17,
  20. Otto Krieger18,
  21. Michael Luebbert19,
  22. Jaroslaw Maciejewski20,
  23. Silvia M.M. Magalhaes21,
  24. Yasushi Miyazaki22,
  25. Michael Pfeilstöcker2,
  26. Mikkael Sekeres20,
  27. Wolfgang R. Sperr15,
  28. Reinhard Stauder23,
  29. Sudhir Tauro24,
  30. Peter Valent15,
  31. Teresa Vallespi25,
  32. Arjan A. van de Loosdrecht26,
  33. Ulrich Germing11, and
  34. Detlef Haase3
  1. 1 Stanford University Cancer Center, Stanford, CA, United States;
  2. 2 Hanusch Hospital, Boltzmann Institute for Leukemia Research, Vienna, Austria;
  3. 3 Georg August Universitaet, Goettingen, Germany;
  4. 4 Hospital Universitario La Fe, Valencia, Spain;
  5. 5 University of Texas, MD Anderson Cancer Center, Houston, TX, United States;
  6. 6 Hospital del Mar, Barcelona, Spain;
  7. 7 University of Rochester Medical Center, James P. Wilmot Cancer Center, Rochester, NY, United States;
  8. 8 St. James's University Hospital, Leeds, United Kingdom;
  9. 9 Hopital Avicenne, Assistance Publique-Hopitaux de Paris(AP-HP)/University Paris XIII, Bobigny, France;
  10. 10 Hopital Cochin, AP-HP/University Paris V, Paris, France;
  11. 11 Heinrich-Heine University Hospital, Duesseldorf, Germany;
  12. 12 Antonio e Biagio e C Arrigo Hospital, Alessandria, Italy;
  13. 13 Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy;
  14. 14 Institute of Hematology and Blood Transfusion, Praha, Czech Republic;
  15. 15 Medical University of Vienna, Vienna, Austria;
  16. 16 The University of Chicago Comprehensive Cancer Research Center, Chicago, IL, United States;
  17. 17 Quest Diagnostics Nichols Institute, Chantilly, VA, United States;
  18. 18 Elisabethinen Hospital, Linz, Austria;
  19. 19 University of Freiburg Medical Center, Freiburg, Germany;
  20. 20 Cleveland Clinic, Cleveland, OH, United States;
  21. 21 Federal University of Ceara, Fortaleza, Brazil;
  22. 22 Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan;
  23. 23 University Hospital of Innsbruck, Innsbruck, Austria;
  24. 24 University of Dundee, Scotland, United Kingdom;
  25. 25 Hospital Universitario Vall d'Hebron, Barcelona, Spain;
  26. 26 VU University Medical Center, Amsterdam, Netherlands
  1. * Corresponding author; email: peterg{at}stanford.edu

Abstract

The International Prognostic Scoring Sytem (IPSS) is an important standard for assessing prognosis of primary untreated adult patients with myelodysplastic syndromes (MDS). To refine the IPSS, MDS patient databases from international institutions were coalesced to assemble a much larger combined database [Revised-IPSS (IPSS-R), n=7012, IPSS, n=816] for analysis. Multiple statistically weighted clinical features were used to generate a prognostic categorization model. Bone marrow cytogenetics, marrow blast percentage and cytopenias remained the basis of the new system. Novel components of the current analysis included: five rather than three cytogenetic prognostic subgroups with specific and new classifications of a number of less common cytogenetic subsets, splitting the low marrow blast percentage value, and depth of cytopenias. This model defined five rather than the four major prognostic categories that are present in the IPSS. Patient age, performance status, serum ferritin and LDH were significant additive features for survival but not for AML transformation. This system comprehensively integrated the numerous known clinical features into a method analyzing MDS patient prognosis more precisely than the initial IPSS. As such, this IPSS-R should prove beneficial for predicting the clinical outcomes of untreated MDS patients and aiding design and analysis of clinical trials in this disease.

  • Submitted March 28, 2012.
  • Accepted June 17, 2012.