Prognostic scoring systems for myelodysplastic syndromes (MDS) in a population‐based setting: a report from the Swedish MDS register

Summary 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 nati...

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Veröffentlicht in:British journal of haematology 2018-06, Vol.181 (5), p.614-627
Hauptverfasser: Moreno Berggren, Daniel, Folkvaljon, Yasin, Engvall, Marie, Sundberg, Johan, Lambe, Mats, Antunovic, Petar, Garelius, Hege, Lorenz, Fryderyk, Nilsson, Lars, Rasmussen, Bengt, Lehmann, Sören, Hellström‐Lindberg, Eva, Jädersten, Martin, Ejerblad, Elisabeth
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container_issue 5
container_start_page 614
container_title British journal of haematology
container_volume 181
creator Moreno Berggren, Daniel
Folkvaljon, Yasin
Engvall, Marie
Sundberg, Johan
Lambe, Mats
Antunovic, Petar
Garelius, Hege
Lorenz, Fryderyk
Nilsson, Lars
Rasmussen, Bengt
Lehmann, Sören
Hellström‐Lindberg, Eva
Jädersten, Martin
Ejerblad, Elisabeth
description Summary 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 100 000 inhabitants. IPSS‐R had a significantly better prognostic power than IPSS (P 
doi_str_mv 10.1111/bjh.15243
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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 100 000 inhabitants. IPSS‐R had a significantly better prognostic power than IPSS (P &lt; 0·001). 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 ≤70 years. 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. 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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. 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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.</abstract><cop>England</cop><pub>Blackwell Publishing Ltd</pub><pmid>29707769</pmid><doi>10.1111/bjh.15243</doi><tpages>14</tpages><orcidid>https://orcid.org/0000-0002-4198-4945</orcidid><oa>free_for_read</oa></addata></record>
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source Wiley Free Content; Wiley Online Library Journals Frontfile Complete
subjects Cytogenetics
Hematology
Incidence
International Prognostic Scoring System
Myelodysplastic syndrome
Population
Population studies
revised International Prognostic Scoring System
Risk assessment
therapy-related myelodysplastic syndrome
WHO Classification-based Prognostic Scoring System
title Prognostic scoring systems for myelodysplastic syndromes (MDS) in a population‐based setting: a report from the Swedish MDS register
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