Cause-specific mortality in individuals with lymphoplasmacytic lymphoma/Waldenstrom 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 center dot 2% for lymphomas, 8 center dot 4% for non-lymphoma cancers and 14 center dot 7% for non-cancer causes for patients aged <65 years at diagnosis of LPL/WM, versus 33 center dot 4%, 11 center dot 2% and 48 center dot 7%, respectively, for those 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 center dot 2, 95% confidence interval [CI] 1 center dot 1-1 center dot 2), most commonly from infectious (n = 188; SMR 1 center dot 8, 95% CI 1 center dot 6-2 center dot 1), respiratory (n = 143; SMR 1 center dot 2, 95% CI 1 center dot 0-1 center dot 4), and digestive (n = 80; SMR 1 center dot 8, 95% CI 1 center dot 4-2 center dot 2) diseases, but no excess mortality from cardiovascular diseases (n = 477, SMR 1 center dot 1, 95% CI 1 center dot 0-1 center dot 1). Risks were highest for non-cancer causes within 1 year of diagnosis (n = 239; SMR<1year 1 center dot 3, 95% CI 1 center dot 2-1 center dot 5), declining thereafter (n = 522; SMR >= 5years 1 center dot 1, 95% CI 1 center dot 1-1 center dot 2). Myelodysplastic syndrome/acute myeloid leukaemia deaths were notably increased (n = 46; SMR 4 center dot 4, 95% CI 3 center dot 2-5 center dot 9), whereas solid neoplasm deaths were only elevated among >= 5-year survivors (n = 145; SMR >= 5years 1 center dot 3, 95% CI 1 center dot 1-1 center dot 5). This work identifies new areas for optimising care and reducing mortality for patients with LPL/WM.