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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

2015; Wiley; Volume: 170; Issue: 3 Linguagem: Inglês

10.1111/bjh.13450

1365-2141

Louise de Swart, Alex Smith, W. Thomas Johnston, Detlef Haase, Jackie Droste, Pierre Fenaux, Argiris Symeonidis, Guillermo Sanz, Eva Hellström‐Lindberg, Jaroslav Čermák, Ulrich Germing, Reinhard Stauder, Otilia Georgescu, Marius MacKenzie, Luca Malcovati, Mette Skov Holm, António Almeida, Krzysztof Mądry, Borhane Slama, Agnès Guerci‐Bresler, Laurence Sanhès, Odile Beyne‐Rauzy, Elisa Luño, David Bowen, Théo de Witte,

Lymphoma Diagnosis and Treatment

Summary 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.