title: 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.
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.
authors.affiliation: Department of Haematology, Radboud University Medical Centre, Nijmegen, The Netherlands.
authors.forename: Louise
authors.initials: L
authors.lastname: de Swart
authors.affiliation: Epidemiology and Cancer Statistics Group, University of York, York, UK.
authors.forename: Alex
authors.initials: A
authors.lastname: Smith
authors.affiliation: Epidemiology and Cancer Statistics Group, University of York, York, UK.
authors.forename: Thomas W
authors.initials: TW
authors.lastname: Johnston
authors.affiliation: Department of Haematology and Oncology, Georg-August-University of Göttingen, Göttingen, Germany.
authors.forename: Detlef
authors.initials: D
authors.lastname: Haase
authors.affiliation: Department of Haematology, Radboud University Medical Centre, Nijmegen, The Netherlands.
authors.forename: Jackie
authors.initials: J
authors.lastname: Droste
authors.affiliation: Service d'Hématologie, Hôpital Saint-Louis, Assistance Publique des Hôpitaux de Paris (AP-HP) and Université Paris 7, Paris, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Fenaux
authors.affiliation: Department of Medicine, Divison of Haematology, University of Patras Medical School, Patras, Greece.
authors.forename: Argiris
authors.initials: A
authors.lastname: Symeonidis
authors.affiliation: Department of Haematology, Hospital Universitario La Fe, Valencia, Spain.
authors.forename: Guillermo
authors.initials: G
authors.lastname: Sanz
authors.affiliation: Department of Medicine, Division of Haematology, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Eva
authors.initials: E
authors.lastname: Hellström-Lindberg
authors.affiliation: Department of Clinical Haematology, Institute of Haematology & Blood Transfusion, Praha, Czech Republic.
authors.forename: Jaroslav
authors.initials: J
authors.lastname: Cermák
authors.affiliation: Department Of Haematology, Oncology and Clinical Immunology, Universitätsklinik Düsseldorf, Düsseldorf, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Germing
authors.affiliation: Department of Internal Medicine V (Haematology and Oncology), Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Stauder
authors.affiliation: Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, Bucharest, Romania.
authors.forename: Otilia
authors.initials: O
authors.lastname: Georgescu
authors.affiliation: Department of Haematology, Radboud University Medical Centre, Nijmegen, The Netherlands.
authors.forename: Marius
authors.initials: M
authors.lastname: MacKenzie
authors.affiliation: Department of Haematology Oncology, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, University of Pavia, Pavia, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Malcovati
authors.affiliation: Department of Haematology, Aarhus University Hospital, Aarhus, Denmark.
authors.forename: Mette S
authors.initials: MS
authors.lastname: Holm
authors.affiliation: Serviço d'Hematologia - Instituto Português de Oncologia de Lisboa, Lisbon, Portugal.
authors.forename: Antonio M
authors.initials: AM
authors.lastname: Almeida
authors.affiliation: Department of Haematology, Oncology and Internal Medicine, Warszawa Medical University, Warszawa, Poland.
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Mądry
authors.affiliation: Service d'Hématologie, Centre Hospitalier d'Avignon, Avignon, France.
authors.forename: Borhane
authors.initials: B
authors.lastname: Slama
authors.affiliation: Service d'Hématologie, Centre Hospitalier Universtaire Brabois Vandoeuvre, Nancy, France.
authors.forename: Agnes
authors.initials: A
authors.lastname: Guerci-Bresler
authors.affiliation: Service d'Hématologie, Centre Hospitalier de Perpignan, Perpignan, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Sanhes
authors.affiliation: Service d'Hématologie, Centre Hospitalier Universitaire de Purpan, Toulouse, France.
authors.forename: Odile
authors.initials: O
authors.lastname: Beyne-Rauzy
authors.affiliation: Servicio d'Hematología, Servicio de Salud del Principado de Asturias, Oviedo, Spain.
authors.forename: Elisa
authors.initials: E
authors.lastname: Luño
authors.affiliation: St. James's Institute of Oncology, Leeds Teaching Hospitals, Leeds, UK.
authors.forename: David
authors.initials: D
authors.lastname: Bowen
authors.affiliation: Department of Tumor Immunology - Nijmegen, Institute for Molecular Life Sciences, Radboud university medical center, Nijmegen, The Netherlands.
authors.forename: Theo
authors.initials: T
authors.lastname: de Witte
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000628:therapy*; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries; D012307:Risk Factors
keywords: International Prognostic Scoring System; co-morbidity score; lower-risk myelodysplastic syndromes; quality of life score; revised International Prognostic Scoring System