Revised International Prognostic Scoring System for Myelodysplastic Syndromes

Peter L. Greenberg(Stanford University), Heinz Tuechler(Hanusch Hospital), Julie Schanz(University of Göttingen), Guillermo Sanz(Hospital Universitari i Politècnic La Fe), Guillermo Garcia‐Manero(The University of Texas MD Anderson Cancer Center), Françesc Solé(Hospital Del Mar), John M. Bennett(University of Rochester Medical Center), David Bowen(St James's University Hospital), Pierre Fenaux(Université Sorbonne Paris Nord), François Dreyfus(Université Paris Cité), Hagop M. Kantarjian(The University of Texas MD Anderson Cancer Center), Andrea Kuendgen(Düsseldorf University Hospital), Alessandro Levis(Azienda Ospedaliera Nazionale SS. Antonio e Biagio e Cesare Arrigo), Luca Malcovati(University of Pavia), Mario Cazzola(University of Pavia), Jaroslav Čermák(Institute of Haematology and Blood Transfusion), Christa Fonatsch(Medical University of Vienna), Michelle M. Le Beau(University of Chicago), Marilyn L. Slovak(Quest Diagnostics (United States)), Otto Krieger(Krankenhaus der Elisabethinen), Michael Luebbert(University of Freiburg), Jaroslaw P. Maciejewski(Cleveland Clinic), Silvia M. M. Magalhães(Universidade Federal do Ceará), Yasushi Miyazaki(Nagasaki University), Michael Pfeilstöcker(Hanusch Hospital), Mikkael A. Sekeres(Cleveland Clinic), Wolfgang R. Sperr(Medical University of Vienna), Reinhard Stauder(University Hospital Innsbruck), Sudhir Tauro(University of Dundee), Peter Valent(Medical University of Vienna), Teresa Vallespı́(Vall d'Hebron Hospital Universitari), Arjan A. van de Loosdrecht(Amsterdam UMC Location Vrije Universiteit Amsterdam), Ulrich Germing(Düsseldorf University Hospital), Detlef Haase(University of Göttingen)
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Abstract

The International Prognostic Scoring System (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: 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.


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