The objective of this study is to externally validate the recently published Revised International Prognostic Scoring System (IPSS-R) for myelodysplastic syndrome (MDS) and compare it with the International Prognostic Scoring System (IPSS). We conducted a retrospective study of 173 adult MDS patients who had not received disease-altering treatment. Using the Cox hazard method, we found the IPSS-R to be a significant predictor of survival (p < 0.001, hazard ratio, HR = 1.82, 95% confidence interval, CI 1.57-2.12) and time to acute myeloid leukemia (AML; p < 0.001, HR = 2.05, 95% CI 1.55-2.70). The IPSS-R has greater prognostic power for survival and time to AML compared with the IPSS, given higher Somers' D values (0.41 vs. 0.39 and 0.55 vs. 0.53, respectively). Using the log-rank test, we found a significant difference when comparing IPSS-R groups (p < 0.02), with the exception of the high-risk versus very high-risk group comparison. The IPSS-R reclassified low-risk and intermediate-1 IPSS groups into four groups (log-rank, p < 0.001) and intermediate-2 and high-risk IPSS groups into three groups (log-rank, p < 0.04, excluding high-risk vs. very high-risk comparison). We conclude that the IPSS-R has significant prognostic utility for MDS patients.

1.
Mufti GJ, Stevens JR, Oscier DG, Hamblin TJ, Machin D: Myelodysplastic syndromes: a scoring system with prognostic significance. Br J Haematol 1985;59:425-433.
2.
Sanz GF, Sanz MA, Vallespi T, Canizo MC, Torrabadella M, Garcia S, Irriguible D, San Miguel JF: Two regression models and a scoring system for predicting survival and planning treatment in myelodysplastic syndromes: a multivariate analysis of prognostic factors in 370 patients. Blood 1989;74:395-408.
3.
Morel P, Hebbar M, Lai JL, Duhamel A, Preudhomme C, Wattel E, Bauters F, Fenaux P: Cytogenetic analysis has strong independent prognostic value in de novo myelodysplastic syndromes and can be incorporated in a new scoring system: a report on 408 cases. Leukemia 1993;7:1315-1323.
4.
Greenberg P, Cox C, LeBeau MM, Fenaux P, Morel P, Sanz G, Sanz M, Vallespi T, Hamblin T, Oscier D, Ohyashiki K, Toyama K, Aul C, Mufti G, Bennett J: International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997;89:2079-2088.
5.
Malcovati L, Germing U, Kuendgen A, Della Porta MG, Pascutto C, Invernizzi R, Giagounidis A, Hildebrandt B, Bernasconi P, Knipp S, Strupp C, Lazzarino M, Aul C, Cazzola M: Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes. J Clin Oncol 2007;25:3503-3510.
6.
Kantarjian H, O'Brien S, Ravandi F, Cortes J, Shan J, Bennett JM, List A, Fenaux P, Sanz G, Issa J-P, Freireich EJ, Garcia-Manero G: Proposal for a new risk model in myelodysplastic syndrome that accounts for events not considered in the original International Prognostic Scoring System. Cancer 2008;113:1351-1361.
7.
Valent P, Horny H, Bennett J, Fonatsch C, Germing U, Greenberg P, Haferlach T, Haase D, Kolb H, Krieger O: Definitions and standards in the diagnosis and treatment of the myelodysplastic syndromes: consensus statements and report from a working conference. Leuk Res 2007;31:727-736.
8.
Greenberg PL, Attar E, Bennett JM, Bloomfield CD, De Castro CM, Deeg HJ, Foran JM, Gaensler K, Garcia-Manero G, Gore SD, Head D, Komrokji R, Maness LJ, Millenson M, Nimer SD, O'Donnell MR, Schroeder MA, Shami PJ, Stone RM, Thompson JE, Westervelt P: NCCN Clinical practice guidelines in oncology: myelodysplastic syndromes. J Natl Compr Canc Netw 2011;9:30-56.
9.
Platzbecker U, Santini V, Mufti GJ, Haferlach C, Maciejewski JP, Park S, Sole F, van de Loosdrecht AA, Haase D: Update on developments in the diagnosis and prognostic evaluation of patients with myelodysplastic syndromes (MDS): consensus statements and report from an expert workshop. Leuk Res 2012;36:264-270.
10.
Park MJ, Kim HJ, Kim SH, Kim DH, Kim SJ, Jang JH, Kim K, Kim WS, Jung CW: Is International Prognostic Scoring System (IPSS) still standard in predicting prognosis in patients with myelodysplastic syndrome? External validation of the WHO Classification-Based Prognostic Scoring System (WPSS) and comparison with IPSS. Eur J Haematol 2008;81:364-373.
11.
Alessandrino EP, Della Porta MG, Bacigalupo A, Van Lint MT, Falda M, Onida F, Bernardi M, Iori AP, Rambaldi A, Cerretti R, Marenco P, Pioltelli P, Malcovati L, Pascutto C, Oneto R, Fanin R, Bosi A: WHO classification and WPSS predict posttransplantation outcome in patients with myelodysplastic syndrome: a study from the Gruppo Italiano Trapianto di Midollo Osseo (GITMO). Blood 2008;112:895-902.
12.
Komrokji RS, Corrales-Yepez M, Al Ali N, Kharfan-Dabaja M, Padron E, Fields T, Lancet JE, List AF: Validation of the MD Anderson Prognostic Risk Model for patients with myelodysplastic syndrome. Cancer 2012;118:2659-2664.
13.
Greenberg PL, Tuechler H, Schanz J, Sanz G, Garcia-Manero G, Sole F, Bennett JM, Bowen D, Fenaux P, Dreyfus F, Kantarjian H, Kuendgen A, Levis A, Malcovati L, Cazzola M, Cermak J, Fonatsch C, Le Beau MM, Slovak ML, Krieger O, Luebbert M, Maciejewski J, Magalhaes SM, Miyazaki Y, Pfeilstocker M, Sekeres M, Sperr WR, Stauder R, Tauro S, Valent P, Vallespi T, van de Loosdrecht AA, Germing U, Haase D: Revised International Prognostic Scoring System (IPSS-R) for myelodysplastic syndromes. Blood 2012;120:2454-2465.
14.
Schanz J, Tuchler H, Sole F, Mallo M, Luno E, Cervera J, Granada I, Hildebrandt B, Slovak ML, Ohyashiki K, Steidl C, Fonatsch C, Pfeilstocker M, Nosslinger T, Valent P, Giagounidis A, Aul C, Lubbert M, Stauder R, Krieger O, Garcia-Manero G, Faderl S, Pierce S, Le Beau MM, Bennett JM, Greenberg P, Germing U, Haase D: New comprehensive cytogenetic scoring system for primary myelodysplastic syndromes (MDS) and oligoblastic acute myeloid leukemia after MDS derived from an international database merge. J Clin Oncol 2012;30:820-829.
15.
Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick HR, Sultan C: Proposals for the classification of the myelodysplastic syndromes. Br J Haematol 1982;51:189-199.
16.
Brunning RD, Orazi R, Germing U, Le Beau MM, Porwit A, Baumann I, Vardiman JW, Hellstrom-Lindberg E: Myelodysplastic syndromes; in: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (ed): WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, ed 4. Lyon, International Agency for Research on Cancer, 2008, pp 87-107.
17.
Shaffer LG, Slovak ML, Campbell LJ: ISCN 2009. Basel, Karger, 2009.
18.
Chun K, Hagemeijer A, Iqbal A, Slovak ML: Implementation of standardized international karyotype scoring practices is needed to provide uniform and systematic evaluation for patients with myelodysplastic syndrome using IPSS criteria: an International Working Group on MDS Cytogenetics Study. Leuk Res 2010;34:160-165.
19.
Thiele J, Kvasnicka HM, Facchetti F, Franco V, van der Walt J, Orazi A: European consensus on grading bone marrow fibrosis and assessment of cellularity. Haematologica 2005;90:1128-1132.
20.
Harrell FE Jr, Lee KL, Mark DB: Multivariable prognostic models: issues in developing models, evaluating assumptions and adequacy, and measuring and reducing errors. Stat Med 1996;15:361-387.
21.
Messa E, Gioia D, Evangelista A, Allione B, Angelucci E, Balleari E, Calabrese M, Catarini M, Centurioni R, Danise P, Del Corso L, Masiera E, Musto P, Pane F, Poloni A, Sanna A, Tassara R, Ciccone G, Levis A, Saglio G, Santini V: High predictive value of the Revised International Prognostic Scoring System (IPSS-R): an external analysis of 646 patients from a multiregional italian MDS registry (abstract). Blood 2012;120:1702a.
22.
Della Porta MG, Malcovati L, Boveri E, Travaglino E, Pietra D, Pascutto C, Passamonti F, Invernizzi R, Castello A, Magrini U, Lazzarino M, Cazzola M: Clinical relevance of bone marrow fibrosis and CD34-positive cell clusters in primary myelodysplastic syndromes. J Clin Oncol 2009;27:754-762.
23.
Mishra A, Al Ali NH, Corrales-Yepez M, Padron E, Zhang L, Epling-Burnette P, Kharfan-Dabaja MA, Pinilla-Ibarz J, Little BJ, Lancet JE, List AF, Komrokji RS: Validation of the Revised International Prognostic Scoring System (R-IPSS) for patients with myelodysplastic syndromes: therapeutic implications (abstract). Blood 2012;120:2816a.
24.
Lamarque M, Raynaud S, Itzykson R, Thepot S, Quesnel B, Dreyfus F, Rauzy OB, Turlure P, Vey N, Recher C, Dartigeas C, Legros L, Delaunay J, Visanica S, Stamatoullas A, Fenaux P, Ades L: The revised IPSS is a powerful tool to evaluate the outcome of MDS patients treated with azacitidine: the GFM experience. Blood 2012;120:5084-5085.
25.
Garcia-Manero G, Shan J, Faderl S, Cortes J, Ravandi F, Borthakur G, Wierda WG, Pierce S, Estey E, Liu J, Huang X, Kantarjian H: A prognostic score for patients with lower risk myelodysplastic syndrome. Leukemia 2008;22:538-543.
26.
Tong WG, Quintás-Cardama A, Kadia T, Borthakur G, Jabbour E, Ravandi F, Faderl S, Wierda W, Pierce S, Shan J, Bueso-Ramos C, Kantarjian H, Garcia-Manero G: Predicting survival of patients with hypocellular myelodysplastic syndrome: development of a disease-specific prognostic score system. Cancer 2012;118:4462-4470.
27.
Bejar R, Stevenson K, Abdel-Wahab O, Galili N, Nilsson B, Garcia-Manero G, Kantarjian H, Raza A, Levine RL, Neuberg D, Ebert BL: Clinical effect of point mutations in myelodysplastic syndromes. N Engl J Med 2011;364:2496-2506.
28.
Bejar R, Stevenson KE, Caughey BA, Abdel-Wahab O, Steensma DP, Galili N, Raza A, Kantarjian H, Levine RL, Neuberg D, Garcia-Manero G, Ebert BL: Validation of a prognostic model and the impact of mutations in patients with lower-risk myelodysplastic syndromes. J Clin Oncol 2012;30:3376-3382.
29.
Papaemmanuil E, Cazzola M, Boultwood J, Malcovati L, Vyas P, Bowen D, Pellagatti A, Wainscoat JS, Hellstrom-Lindberg E, Gambacorti-Passerini C, Godfrey AL, Rapado I, Cvejic A, Rance R, McGee C, Ellis P, Mudie LJ, Stephens PJ, McLaren S, Massie CE, Tarpey PS, Varela I, Nik-Zainal S, Davies HR, Shlien A, Jones D, Raine K, Hinton J, Butler AP, Teague JW, Baxter EJ, Score J, Galli A, Della Porta MG, Travaglino E, Groves M, Tauro S, Munshi NC, Anderson KC, El-Naggar A, Fischer A, Mustonen V, Warren AJ, Cross NC, Green AR, Futreal PA, Stratton MR, Campbell PJ: Somatic SF3B1 mutation in myelodysplasia with ring sideroblasts. N Engl J Med 2011;365:1384-1395.
30.
Wells DA, Benesch M, Loken MR, Vallejo C, Myerson D, Leisenring WM, Deeg HJ: Myeloid and monocytic dyspoiesis as determined by flow cytometric scoring in myelodysplastic syndrome correlates with the IPSS and with outcome after hematopoietic stem cell transplantation. Blood 2003;102:394-403.
31.
van de Loosdrecht AA, Westers TM, Westra AH, Drager AM, van der Velden VH, Ossenkoppele GJ: Identification of distinct prognostic subgroups in low- and intermediate-1-risk myelodysplastic syndromes by flow cytometry. Blood 2008;111:1067-1077.
32.
Della Porta MG, Malcovati L, Strupp C, Ambaglio I, Kuendgen A, Zipperer E, Travaglino E, Invernizzi R, Pascutto C, Lazzarino M, Germing U, Cazzola M: Risk stratification based on both disease status and extra-hematologic comorbidities in patients with myelodysplastic syndrome. Haematologica 2011;96:441-449.
33.
Naqvi K, Garcia-Manero G, Sardesai S, Oh J, Vigil CE, Pierce S, Lei X, Shan J, Kantarjian HM, Suarez-Almazor ME: Association of comorbidities with overall survival in myelodysplastic syndrome: development of a prognostic model. J Clin Oncol 2011;29:2240-2246.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.