title: Trends in incidence, initial treatment and survival of myelodysplastic syndromes: a population-based study of 5144 patients diagnosed in the Netherlands from 2001 to 2010.
abstract: BACKGROUND
  Studies with long-term follow-up of patients with myelodysplastic syndromes (MDS) based on data from nationwide population-based cancer registries are lacking. We conducted a nationwide population-based study to assess trends in incidence, initial treatment and survival in MDS patients diagnosed in the Netherlands from 2001 to 2010.
  
  
  METHODS
  We identified 5144 MDS patients (median age, 74 years) from the Netherlands Cancer Registry (NCR). The NCR only includes MDS cases that were confirmed by bone marrow examinations. Information regarding initial treatment decisions was available in the NCR.
  
  
  RESULTS
  The age-standardised incidence rate of MDS was 2.3/100,000 in 2001-2005 and 2.8/100,000 in 2006-2010. The incidence increased with older age, with the highest incidence among those aged ≥80 years (32.1/100,000 in 2006-2010). Forty-nine percent of all MDS cases were unspecified. Of all patients, 89% receive no treatment or only supportive care and 8% were started on intensive therapy as initial treatment. Survival did not improve over time. The 5-year relative survival was 53%, 58%, 48%, 38% and 18% in patients with refractory anaemia (RA), RA with ringed sideroblasts, 5q-syndrome, refractory cytopenia with multilineage dysplasia, and RA with excess blasts, respectively.
  
  
  CONCLUSIONS
  The incidence of MDS increased over time due to improved notification and better disease awareness, and has stabilised since 2007. The classification of MDS seems challenging as almost half of the pathologically confirmed cases were unspecified. The lack of improvement in survival might be explained by the limited availability of therapeutic agents. Therefore, ameliorated management and new treatment options are warranted.
authors.affiliation: Department of Hematology, Erasmus MC Cancer Institute, Erasmus University Medical Center, Rotterdam, The Netherlands.
authors.forename: Avinash G
authors.initials: AG
authors.lastname: Dinmohamed
authors.affiliation: Department of Registration and Research, Comprehensive Cancer Centre The Netherlands, Utrecht, The Netherlands.
authors.forename: Otto
authors.initials: O
authors.lastname: Visser
authors.affiliation: Clinical Trial Center, Erasmus MC Cancer Institute, Erasmus University Medical Center, Rotterdam, The Netherlands.
authors.forename: Yvette
authors.initials: Y
authors.lastname: van Norden
authors.affiliation: Department of Hematology, VU University Medical Center, Cancer Center Amsterdam, Amsterdam, The Netherlands.
authors.forename: Peter C
authors.initials: PC
authors.lastname: Huijgens
authors.affiliation: Department of Hematology, Erasmus MC Cancer Institute, Erasmus University Medical Center, Rotterdam, The Netherlands.
authors.forename: Pieter
authors.initials: P
authors.lastname: Sonneveld
authors.affiliation: Department of Hematology, VU University Medical Center, Cancer Center Amsterdam, Amsterdam, The Netherlands.
authors.forename: Arjan A
authors.initials: AA
authors.lastname: van de Loosdrecht
authors.affiliation: Department of Hematology, Erasmus MC Cancer Institute, Erasmus University Medical Center, Rotterdam, The Netherlands. Electronic address: m.lavrencic@erasmusmc.nl.
authors.forename: Mojca
authors.initials: M
authors.lastname: Jongen-Lavrencic
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D003131:Combined Modality Therapy; D004358:Drug Therapy / Q000379:methods; D005260:Female; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D009426:Netherlands / Q000453:epidemiology; D011159:Population Surveillance / Q000379:methods*; D012042:Registries / Q000706:statistics & numerical data*; D033581:Stem Cell Transplantation / Q000379:methods; D016896:Treatment Outcome
keywords: Epidemiology; Incidence; Myelodysplastic syndromes; Population-based; Registry; Survival; Treatment