Revised International Prognostic Scoring System for Myelodysplastic Syndromes

Author:

Greenberg Peter L.1,Tuechler Heinz2,Schanz Julie3,Sanz Guillermo4,Garcia-Manero Guillermo5,Solé Francesc6,Bennett John M.7,Bowen David8,Fenaux Pierre9,Dreyfus Francois10,Kantarjian Hagop5,Kuendgen Andrea11,Levis Alessandro12,Malcovati Luca13,Cazzola Mario13,Cermak Jaroslav14,Fonatsch Christa15,Le Beau Michelle M.16,Slovak Marilyn L.17,Krieger Otto18,Luebbert Michael19,Maciejewski Jaroslaw20,Magalhaes Silvia M. M.21,Miyazaki Yasushi22,Pfeilstöcker Michael2,Sekeres Mikkael20,Sperr Wolfgang R.15,Stauder Reinhard23,Tauro Sudhir24,Valent Peter15,Vallespi Teresa25,van de Loosdrecht Arjan A.26,Germing Ulrich11,Haase Detlef3

Affiliation:

1. Stanford University Cancer Center, Stanford, CA;

2. Hanusch Hospital, Boltzmann Institute for Leukemia Research, Vienna, Austria;

3. Georg August Universität, Göttingen, Germany;

4. Hospital Universitario La Fe, Valencia, Spain;

5. The University of Texas, MD Anderson Cancer Center, Houston, TX;

6. Hospital del Mar, Barcelona, Spain;

7. James P. Wilmont Cancer Center, University of Rochester Medical Center, Rochester, NY;

8. St James's University Hospital, Leeds, United Kingdom;

9. Hôpital Avicenne, Assistance Publique–Hôpitaux de Paris (AP-HP)/University Paris XIII, Bobigny, France;

10. Hôpital Cochin, AP-HP University of Paris V, Paris, France;

11. Heinrich-Heine University Hospital, Düsseldorf, Germany;

12. Antonio e Biagio e C Arrigo Hospital, Alessandria, Italy;

13. Fondazione Istituti di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo and University of Pavia, Pavia, Italy;

14. Institute of Hematology and Blood Transfusion, Praha, Czech Republic;

15. Medical University of Vienna, Vienna, Austria;

16. University of Chicago Comprehensive Cancer Research Center, Chicago, IL;

17. Quest Diagnostics Nichols Institute, Chantilly, VA;

18. Elisabethinen Hospital, Linz, Austria;

19. University of Freiburg Medical Center, Freiburg, Germany;

20. Cleveland Clinic, Cleveland, OH;

21. Federal University of Ceara, Fortaleza, Brazil;

22. Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan;

23. University Hospital of Innsbruck, Innsbruck, Austria;

24. University of Dundee, Scotland, United Kingdom;

25. Hospital Universitario Vall d'Hebron, Barcelona, Spain; and

26. VU University Medical Center, Amsterdam, The Netherlands

Abstract

AbstractThe International Prognostic Scoring Sytem (IPSS) is an important standard for ssessing 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: 5 rather than 3 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 5 rather than the 4 major prognostic categories that are present in the IPSS. Patient age, performance status, serum ferritin, and lactate dehydrogenase were significant additive features for survival but not for acute myeloid leukemia 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.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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