Primary therapy and relative survival in patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia: a population-based study in the Netherlands, 1989-2018

Br J Haematol. 2022 Feb;196(3):660-669. doi: 10.1111/bjh.17856. Epub 2021 Oct 3.

Abstract

It is unclear how treatment advances impacted the population-level survival of patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia (LPL/WM). Therefore, we assessed trends in first-line therapy and relative survival (RS) among patients with LPL/WM diagnosed in the Netherlands between 1989 and 2018 (N = 6232; median age, 70 years; 61% males) using data from the nationwide Netherlands Cancer Registry. Patients were grouped into three age groups (<65, 66-75 and >75 years) and four calendar periods. Overall, treatment with anti-neoplastic agents within 1 year post-diagnosis gradually decreased over time, following a broader application of an initial watch-and-wait approach. Approximately 40% of patients received anti-neoplastic therapy during 2011-2018. Furthermore, use of chemotherapy alone decreased over time, following an increased application of chemoimmunotherapy. Detailed data among 1596 patients diagnosed during 2014-2018 revealed that dexamethasone-rituximab-cyclophosphamide was the most frequently applied regimen; its use increased from 14% to 39% between 2014 and 2018. The 5-year RS increased significantly over time, particularly since the introduction of rituximab in the early-mid 2000s. The 5-year RS during 1989-1995 was 75%, 65%, and 46% across the age groups compared to 93%, 85%, and 79% during 2011-2018. However, the survival improvement was less pronounced after 2011. Collectively, the impressive survival improvement may be accounted for by broader application of rituximab-containing therapy. The lack of survival improvement in the post-rituximab era warrants studies across multiple lines of therapy to further improve survival in LPL/WM.

Keywords: Waldenström macroglobulinaemia; lymphoplasmacytic lymphoma; population-based study; relative survival.

Publication types

  • Historical Article

MeSH terms

  • Aged
  • Aged, 80 and over
  • Combined Modality Therapy
  • Disease Management
  • Female
  • History, 20th Century
  • History, 21st Century
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Mortality
  • Netherlands / epidemiology
  • Prognosis
  • Public Health Surveillance
  • Registries
  • Treatment Outcome
  • Waldenstrom Macroglobulinemia / diagnosis
  • Waldenstrom Macroglobulinemia / history
  • Waldenstrom Macroglobulinemia / mortality*
  • Waldenstrom Macroglobulinemia / therapy*