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 versus 33·4%, 11·2% and 48·7%, 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] 1·2, 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 _{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.}