Blood Res 2018; 53(3): 189-197  https://doi.org/10.5045/br.2018.53.3.189
Clinical features and survival outcomes of patients with lymphoplasmacytic lymphoma, including non-IgM type, in Korea: a single-center experience
Jihoon Kang, Jung Yong Hong, Cheolwon Suh
Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
Correspondence to: Cheolwon Suh, M.D., Ph.D. Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea E-mail: csuh@amc.seoul.kr
Received: December 24, 2017; Revised: February 12, 2018; Accepted: February 22, 2018; Published online: September 30, 2018.
© The Korean Journal of Hematology. All rights reserved.

cc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Background
The incidence of lymphoplasmacytic lymphoma (LPL) is lower in Asian than in Western populations. Few studies have described the clinical features and treatment outcomes of patients with LPL, including non-IgM LPL, in East Asia.
Methods
We retrospectively analyzed patients diagnosed with LPL at Asan Medical Center between January 2001 and March 2016. We evaluated the clinical features and survival outcomes of patients with LPL and non-IgM LPL and compared these data with those of patients with LPL/Waldenström’s macroglobulinemia (WM).
Results
The median age at diagnosis of patients with LPL was 61.5 years (range, 34‒77 yr); most patients were male (91%). Approximately three-quarters of the 22 patients with LPL were in the low or intermediate risk groups according to the International Prognostic Scoring System for Waldenström’s Macroglobulinemia classification. The median follow-up duration was 75 months [95% confidence interval (CI), 48‒102 mo], and the median overall survival (OS) was 81 months (95% CI, 0‒167 mo). The number of patients in the non-IgM LPL group who exhibited extramedullary involvement was higher than in the LPL/WM group. OS of the LPL/WM group was improved compared with that of the non-IgM LPL group [median not reached vs. 10.0 mo (95% CI, 0‒36.7); P=0.05].
Conclusion
We present a single-center experience of 22 patients with LPL, including a non-IgM co-hort, in Korea. The treatment of non-IgM LPL was heterogeneous, and patients with non-IgM LPL showed a higher 5-year mortality rate and more adverse prognostic factors than those with LPL/WM.
Keywords: Lymphoplasmacytic lymphoma, Non-IgM, Waldenström’s macroglobulinemia


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