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Br J Haematol. 2015 Aug;170(3):372-83. doi: 10.1111/bjh.13450. Epub 2015 Apr 24.

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.

Author information

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 Göttingen, Göttingen, Germany.
4
Service d'Hématologie, Hôpital Saint-Louis, Assistance Publique des Hôpitaux 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, Universitätsklinik 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
Serviço 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:

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

PMID:
25907546
DOI:
10.1111/bjh.13450
[Indexed for MEDLINE]

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