Incidence, prevalence, mortality, and causes of death in Waldenström macroglobulinemia: a nationwide, population-based cohort study

Abstract

Background: The epidemiological features of Waldenström macroglobulinemia (WM) have seldom been investigated at a national level, particularly in East Asia. The goal of our study is to present the incidence, prevalence, mortality, survival with competing risks, and causes of death of patients with WM. Methods: We used a national population-based database, operated by the Health Insurance Review and Assessment Service of the Korean government. This data includes information on all WM patients diagnosed according to uniform criteria, between 2003 and 2016. Results: The total number of patients newly diagnosed with WM during the study period was 427, with a male-tofemale ratio of 3.2:1. The incidence increased from 0.03 to 0.10 per 10 5 between 2003 and 2016, and the prevalence was 0.42 per 10 5 in 2016. A total of 217 patients with WM died during the study period (standardized mortality ratio = 7.57), and the overall survival (OS) of WM patients was 47.5%. On multivariate analysis, older age was associated with worse OS (P < 0.0001). WM was the most common cause of death (n = 102, 48.6%), followed by other malignant neoplasms (n = 82, 39.0%). Conclusions: The national incidence of WM in Korea, a racially homogeneous country in Asia, was lower than that in previous reports from other countries, reflecting ethnic disparities. However, the incidence increased, and mortality was the highest ever reported. The main cause of death was WM in itself. This study reflects the need for greater awareness of WM, particularly in Asian countries.

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

“…3,21,22 In two other populationbased studies, the incidence initially increases until the late 1990s/early 2000s, after which it stabilised. 6,23 Unlike these studies, an increase in incidence was not observed in our present study. This difference could be due to stable registration and diagnostic practices in the Netherlands.…”

“…In the present large, nationwide, population‐based study among patients diagnosed with LPL/WM during a 30‐year period in the Netherlands, we demonstrated trends in applying different first‐line therapies over time and improving RS among all age groups after the introduction of rituximab. The present study complements and extends on the limited epidemiological studies performed in LPL/WM 3,19,21–24,31 . Moreover, the present study is, to the best of our knowledge, the only population‐based study to date that includes pathology confirmation in LPL/WM that offers comprehensive information on incidence, primary therapy and RS from a historical and contemporary perspective.…”

“…The present study complements and extends on the limited epidemiological studies performed in LPL/WM. 3,19,[21][22][23][24]31 Moreover, the present study is, to the best of our knowledge, the only population-based study to date that includes pathology confirmation in LPL/WM that offers comprehensive information on incidence, primary therapy and RS from a historical and contemporary perspective. The second-largest population based-study in the USA lacked pathology confirmation and did not report on primary therapy.…”

“…At present, large, population‐based studies that assess the outcomes of patients with LPL/WM are scarce. Furthermore, these studies are mostly outdated and usually lack comprehensive information on incidence, therapy and survival 3,19–24 . Therefore, it is unclear how contemporary treatment advances in LPL/WM have impacted the population‐level survival.…”

Conditional relative survival in Waldenström’s macroglobulinaemia: a population‐based study in The Netherlands

“…The few available population-based studies in LPL/WM revealed that the introduction of rituximab to the therapeutic arsenal of LPL/WM improved the population-level survival of LPL/WM patients. [2][3][4][5][6][7] These studies present survival estimates measured from diagnosis, which is helpful to inform on the prognosis for newly diagnosed patients. However, these estimates can be somewhat discouraging due to patients who die within the first years post diagnosis.…”