Validation of the revised international prognostic scoring system (IPSS-R) in patients with lower-risk myelodysplastic syndromes: a report from the prospective European LeukaemiaNet MDS (EUMDS) registry

Louise de Swart, Alex Smith, Thomas W. Johnston, Detlef Haase, Jackie Droste, Pierre Fenaux, Argiris Symeonidis, Guillermo Sanz, Eva Hellström-Lindberg, Jaroslav Cermák, Ulrich Germing, Reinhard Stauder, Otilia Georgescu, Marius MacKenzie, Luca Malcovati, Mette S. Holm, Antonio M. Almeida, Krzysztof Mądry, Borhane Slama, Agnes Guerci-BreslerLaurence Sanhes, Odile Beyne-Rauzy, Elisa Luño, David Bowen, Theo de Witte

Research output: Contribution to journalArticle

Abstract

Baseline characteristics, disease-management and outcome of 1000 lower-risk myelodysplastic syndrome (MDS) patients within the European LeukaemiaNet MDS (EUMDS) Registry are described in conjunction with the validation of the revised International Prognostic Scoring System (IPSS-R). The EUMDS registry confirmed established prognostic factors, such as age, gender and World Health Organization 2001 classification. Low quality of life (EQ-5D visual analogue scale score) was significantly associated with reduced survival. A high co-morbidity index predicted poor outcome in univariate analyses. The IPSS-R identified a large group of 247 patients with Low (43%) and Very low (23%) risk score within the IPSS intermediate-1 patients. The IPSS-R also identified 32 High or Very high risk patients within the IPSS intermediate-1 patients. IPSS-R was superior to the IPSS for predicting both disease progression and survival. Seventy percent of patients received MDS-specific treatment or supportive care, including red blood cell transfusions (51%), haematopoietic growth factors (58%) and iron chelation therapy (8%), within 2 years of diagnosis; while 30% of the patients only required active monitoring. The IPSS-R proved its utility as a more refined risk stratification tool for the identification of patients with a very good or poor prognosis and in this lower-risk MDS population.

Original languageEnglish
Pages (from-to)372-383
Number of pages12
JournalBritish Journal of Haematology
Volume170
Issue number3
DOIs
Publication statusPublished - Aug 1 2015

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Keywords

  • co-morbidity score
  • International Prognostic Scoring System
  • lower-risk myelodysplastic syndromes
  • quality of life score
  • revised International Prognostic Scoring System

ASJC Scopus subject areas

  • Medicine(all)

Cite this

de Swart, L., Smith, A., Johnston, T. W., Haase, D., Droste, J., Fenaux, P., Symeonidis, A., Sanz, G., Hellström-Lindberg, E., Cermák, J., Germing, U., Stauder, R., Georgescu, O., MacKenzie, M., Malcovati, L., Holm, M. S., Almeida, A. M., Mądry, K., Slama, B., ... de Witte, T. (2015). Validation of the revised international prognostic scoring system (IPSS-R) in patients with lower-risk myelodysplastic syndromes: a report from the prospective European LeukaemiaNet MDS (EUMDS) registry. British Journal of Haematology, 170(3), 372-383. https://doi.org/10.1111/bjh.13450