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Revised International Prognostic Scoring System for Myelodysplastic Syndromes

Blood · 2012 · Vol. 120(12) · pp. 2454–2465
Peter L. GreenbergHeinz TuechlerJulie SchanzGuillermo SanzGuillermo Garcia‐ManeroFrançesc SoléJohn M. BennettDavid BowenPierre FenauxFrançois DreyfusHagop M. KantarjianAndrea KuendgenAlessandro LevisLuca MalcovatiMario CazzolaJaroslav ČermákChrista FonatschMichelle M. Le BeauMarilyn L. SlovakOtto KriegerMichael LuebbertJaroslaw P. MaciejewskiSilvia M. M. MagalhãesYasushi MiyazakiMichael PfeilstöckerMikkael A. SekeresWolfgang R. SperrReinhard StauderSudhir TauroPeter ValentTeresa Vallespı́Arjan A. van de LoosdrechtUlrich GermingDetlef Haase

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.

Acute Myeloid Leukemia ResearchMyeloproliferative Neoplasms: Diagnosis and TreatmentHematological disorders and diagnosticsInternational Prognostic Scoring SystemMyelodysplastic syndromesMedicineInternal medicineOncologyBone marrowAnemia

MeSH terms

AdultAgedAged, 80 and overBone MarrowChromosome AberrationsFemaleHumansInternational AgenciesKaryotypingMaleMiddle AgedMyelodysplastic SyndromesPancytopeniaPrognosisRisk Factors

Funding

  • Amgen
  • Celgene
  • Fondazione Cariplo
  • Associazione Italiana per la Ricerca sul Cancro
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