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Prognostic scoring systems for myelodysplastic syndromes (MDS) in a population-based setting: a report from the Swedish MDS register
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2018 (Engelska)Ingår i: British Journal of Haematology, ISSN 0007-1048, E-ISSN 1365-2141, Vol. 181, nr 5, s. 614-627Artikel i tidskrift (Refereegranskat) Published
Abstract [en]

The myelodysplastic syndromes (MDS) have highly variable outcomes and prognostic scoring systems are important tools for risk assessment and to guide therapeutic decisions. However, few population-based studies have compared the value of the different scoring systems. With data from the nationwide Swedish population-based MDS register we validated the International Prognostic Scoring System (IPSS), revised IPSS (IPSS-R) and the World Health Organization (WHO) Classification-based Prognostic Scoring System (WPSS). We also present population-based data on incidence, clinical characteristics including detailed cytogenetics and outcome from the register. The study encompassed 1329 patients reported to the register between 2009 and 2013, 14% of these had therapy-related MDS (t-MDS). Based on the MDS register, the yearly crude incidence of MDS in Sweden was 2.9 per 100000 inhabitants. IPSS-R had a significantly better prognostic power than IPSS (P < 0001). There was a trend for better prognostic power of IPSS-R compared to WPSS (P=0.05) and for WPSS compared to IPSS (P=0.07). IPSS-R was superior to both IPSS and WPSS for patients aged <= 70years. Patients with t-MDS had a worse outcome compared to de novo MDS (d-MDS), however, the validity of the prognostic scoring systems was comparable for d-MDS and t-MDS. In conclusion, population-based studies are important to validate prognostic scores in a real-world' setting. In our nationwide cohort, the IPSS-R showed the best predictive power.

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2018. Vol. 181, nr 5, s. 614-627
Nyckelord [en]
myelodysplastic syndrome, International Prognostic Scoring System, revised International Prognostic Scoring System, WHO Classification-based Prognostic Scoring System, therapy-related myelodysplastic syndrome
Nationell ämneskategori
Hematologi
Identifikatorer
URN: urn:nbn:se:umu:diva-150884DOI: 10.1111/bjh.15243ISI: 000433333100007PubMedID: 29707769Scopus ID: 2-s2.0-85046032311OAI: oai:DiVA.org:umu-150884DiVA, id: diva2:1244269
Tillgänglig från: 2018-08-31 Skapad: 2018-08-31 Senast uppdaterad: 2019-05-10Bibliografiskt granskad

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Lorenz, Fryderyk

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