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期刊界 All Journals 搜尽天下杂志传播学术成果专业期刊搜索期刊信息化学术搜索

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

Authors:	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‐Bresler Laurence Sanhes Odile Beyne‐Rauzy Elisa Luño David Bowen Theo de Witte

Affiliation:	1. Department of Haematology, Radboud University Medical Centre, Nijmegen, The Netherlands;2. Epidemiology and Cancer Statistics Group, University of York, York, UK;3. Department of Haematology and Oncology, Georg‐August‐University of G?ttingen, G?ttingen, Germany;4. Service d'Hématologie, H?pital Saint‐Louis, Assistance Publique des H?pitaux de Paris (AP‐HP) and Université Paris 7, Paris, France;5. Department of Medicine, Divison of Haematology, University of Patras Medical School, Patras, Greece;6. Department of Haematology, Hospital Universitario La Fe, Valencia, Spain;7. Department of Medicine, Division of Haematology, Karolinska Institutet, Stockholm, Sweden;8. Department of Clinical Haematology, Institute of Haematology & Blood Transfusion, Praha, Czech Republic;9. Department Of Haematology, Oncology and Clinical Immunology, Universit?tsklinik Düsseldorf, Düsseldorf, Germany;10. Department of Internal Medicine V (Haematology and Oncology), Innsbruck Medical University, Innsbruck, Austria;11. Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, Bucharest, Romania;12. Department of Haematology Oncology, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, University of Pavia, Pavia, Italy;13. Department of Haematology, Aarhus University Hospital, Aarhus, Denmark;14. Servi?o d'Hematologia ‐ Instituto Português de Oncologia de Lisboa, Lisbon, Portugal;15. Department of Haematology, Oncology and Internal Medicine, Warszawa Medical University, Warszawa, Poland;16. Service d'Hématologie, Centre Hospitalier d'Avignon, Avignon, France;17. Service d'Hématologie, Centre Hospitalier Universtaire Brabois Vandoeuvre, Nancy, France;18. Service d'Hématologie, Centre Hospitalier de Perpignan, Perpignan, France;19. Service d'Hématologie, Centre Hospitalier Universitaire de Purpan, Toulouse, France;20. Servicio d'Hematología, Servicio de Salud del Principado de Asturias, Oviedo, Spain;21. St. James's Institute of Oncology, Leeds Teaching Hospitals, Leeds, UK;22. Department of Tumor Immunology ‐ Nijmegen, Institute for Molecular Life Sciences, Radboud university medical center, Nijmegen, The Netherlands

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.

Keywords:	lower‐risk myelodysplastic syndromes International Prognostic Scoring System revised International Prognostic Scoring System co‐morbidity score quality of life score

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