Cause-specific mortality in individuals with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia, 2000-2016.

Abstract

Data on cause-specific mortality after lymphoplasmacytic lymphoma (LPL) and Waldenström 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 <65years at diagnosis of LPL/WM, versus 33·4%, 11·2% and 48·7%, respectively, for those aged ≥75years. 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 1year of diagnosis (n=239; SMR<1year 1·3, 95% CI 1·2-1·5), declining thereafter (n=522; 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.