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

Abstract

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 < 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. In our nationwide cohort, the IPSS-R showed the best predictive power.

Prognostic scoring systems and comorbidities in chronic myelomonocytic leukaemia: a nationwide population‐based study

“…The 5‐year cumulative progression to acute myeloid leukaemia (AML) is reported to be 21–29% 8,9 . Therapy‐related CMML (t‐CMML) is in accordance with therapy‐related MDS reported to have more high‐risk cytogenetics and a shorter overall survival (OS) 10–12 …”

“…The Swedish nationwide MDS register includes patients with MDS but also MDS/MPN, where CMML is the largest group. The register is described in detail elsewhere 10 . For the time period of this study the coverage of the MDS register against the Swedish Cancer Register was 97%.…”

Asian Population Is More Prone to Develop High-Risk Myelodysplastic Syndrome, Concordantly with Their Propensity to Exhibit High-Risk Cytogenetic Aberrations

“…It is based on five cytogenetic groups, in addition to the definitions of the depth of cytopenias and of bone marrow blast infiltration. For this reason, we only considered articles based on IPSS-R criteria [26,[36][37][38][39]. Previous reports demonstrate that ethnic differences may exist in the prognosis of MDS patients in scoring category [28,40].…”

“…Previous reports demonstrate that ethnic differences may exist in the prognosis of MDS patients in scoring category [28,40]. Here we collected and compared the prognosis scoring for MDS based on IPSS-R from different world areas [26,29,31,36,39,[41][42][43][44][45][46][47] (Figures 3 and 5, Table S4). Collectively, the sizes of Western and Asian cohorts are respectively 16,432 and 1458 patients (Table S4).…”

“…Collectively, the sizes of Western and Asian cohorts are respectively 16,432 and 1458 patients (Table S4). [26,29,31,36,39,[41][42][43][44][45][46][47]. Raw data and statistical analysis are presented in Supplemental Table S4.…”