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.

Fiche publication


Date publication

août 2015

Auteurs

Membres identifiés du Cancéropôle Est :
Dr GUERCI-BRESLER Agnès


Tous les auteurs :
de Swart L, Smith A, Johnston TW, Haase D, Droste J, Fenaux P, Symeonidis A, Sanz G, Hellstrom-Lindberg E, Cermak J, Germing U, Stauder R, Georgescu O, MacKenzie M, Malcovati L, Holm MS, Almeida AM, Madry K, Slama B, Guerci-Bresler A, Sanhes L, Beyne-Rauzy O, Luno E, Bowen D, de Witte T

Résumé

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.

Référence

Br J Haematol. 2015 Aug;170(3):372-83