Cause‐specific mortality in individuals with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia, 2000–2016

Data on cause-specific mortality after lymphoplasmacytic lymphoma (LPL) and Waldenstrom macroglobulinaemia (WM) are lacking. We identified causes of death amongst 7289 adults diagnosed with incident first primary LPL (n = 3108) or WM (n = 4181) during 2000-2016 in 17 USA population-based cancer registries. Based on 3132 deaths, 16-year cumulative mortality was 23.2% for lymphomas, 8.4% for non-lymphoma cancers and 14.7% for non-cancer causes for patients aged /=75 years. Compared with the general population, patients with LPL/WM had a 20% higher risk of death due to non-cancer causes (n = 1341 deaths, standardised mortality ratio [SMR] 1.2, 95% confidence interval [CI] 1.1-1.2), most commonly from infectious (n = 188; SMR 1.8, 95% CI 1.6-2.1), respiratory (n = 143; SMR 1.2, 95% CI 1.0-1.4), and digestive (n = 80; SMR 1.8, 95% CI 1.4-2.2) diseases, but no excess mortality from cardiovascular diseases (n = 477, SMR 1.1, 95% CI 1.0-1.1). Risks were highest for non-cancer causes within 1 year of diagnosis (n = 239; SMR /=5years 1.1, 95% CI 1.1-1.2). Myelodysplastic syndrome/acute myeloid leukaemia deaths were notably increased (n = 46; SMR 4.4, 95% CI 3.2-5.9), whereas solid neoplasm deaths were only elevated among >/=5-year survivors (n = 145; SMR>/=5years 1.3, 95% CI 1.1-1.5). This work identifies new areas for optimising care and reducing mortality for patients with LPL/WM.