Abstract
BackgroundThe 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.MethodsWe 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.ResultsThe total number of patients newly diagnosed with WM during the study period was 427, with a male-to-female ratio of 3.2:1. The incidence increased from 0.03 to 0.10 per 105 between 2003 and 2016, and the prevalence was 0.42 per 105 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%).ConclusionsThe 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.
Highlights
The epidemiological features of Waldenström macroglobulinemia (WM) have seldom been investigated at a national level, in East Asia
Incidence of WM The overall incidence and the age- and gender-specific annual incidence of WM are presented in Table 1 and Fig. 1a
The highest incidence rate of WM was observed in people aged between 75 and 79 years (8.15 per 100,000 individuals), accounting for 19.7% of all incident cases
Summary
The epidemiological features of Waldenström macroglobulinemia (WM) have seldom been investigated at a national level, in East Asia. Waldenström macroglobulinemia (WM) is an indolent B-cell malignancy characterized by the presence of immunoglobulin M (IgM) monoclonal gammopathy and lymphoplasmacytic bone marrow infiltration [1]. Owing to the diverse clinical course of WM, treatment is not necessary for some patients at diagnosis. Most patients with WM require systemic treatment at some time during the course of their disease [4]. Jeong et al BMC Cancer (2020) 20:623 monoclonal antibodies, proteasome inhibitors, and Bruton tyrosine kinase inhibitors, with better understanding of the pathogenesis, have improved the expected clinical outcomes of WM [5, 6]. WM still remains incurable, and its management is becoming increasingly complex [3, 4]
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