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

doi:10.5045/br.2018.53.3.189

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Blood Res 2018; 53(3):

Published online September 28, 2018

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, and Cheolwon Suh^*

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Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Correspondence to : 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. csuh@amc.seoul.kr

Received: December 24, 2017; Revised: February 12, 2018; Accepted: February 22, 2018

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

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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 cohort, 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

Article

Original Article

Blood Res 2018; 53(3): 189-197

Published online September 28, 2018 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, and Cheolwon Suh^*

Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Correspondence to: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. csuh@amc.seoul.kr

Received: December 24, 2017; Revised: February 12, 2018; Accepted: February 22, 2018

Abstract

Background

Methods

Results

Conclusion

Keywords: Lymphoplasmacytic lymphoma, Non-IgM, Waldenström's macroglobulinemia

Abstract

Fig 1.

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Figure 1.

Overall survival (A) and progression-free survival (B) curves in overall lymphoplasmacytic lymphoma patients.

Blood Research 2018; 53: 189-197https://doi.org/10.5045/br.2018.53.3.189

Fig 2.

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Figure 2.

Overall survival (A) and progression-free survival (B) curves in patients with lymphoplasmacytic lymphoma (LPL)/Waldenström's macroglobulinemia (WM) and non-IgM LPL.

Blood Research 2018; 53: 189-197https://doi.org/10.5045/br.2018.53.3.189

Table 1 Baseline patient characteristics.

^a)One patient with LPL/WM was excluded because of missing data for β2-MG. ^b)Two patients with LPL/WM were excluded because of missing data for cytogenetics..

Abbreviations: β2-MG, beta2-microglobulin; BM, bone marrow; ECOG PS, Eastern Cooperative Oncology Group Performance Status; ISSWM, International Prognostic Scoring System for Waldenström's Macroglobulinemia; LDH, lactate dehydrogenase; LPL, lymphoplasmacytic lymphoma; WM, Waldenström's macroglobulinemia..

Table 2 Outcomes and treatments of patients.

Abbreviations: BM, bone marrow; GIT, gastrointestinal tract; LN, lymph node; LPL, lymphoplasmacytic lymphoma; OS, overall survival; BR, bendamustine+rituximab; R, rituximab; CHOP, cyclophosphamide+doxorubicin+vincristine+prednisolone; C(V)P, cyclophosphamide+(vincristine)+prednisolone; FDR, fludarabine; HD-dex, high-dose dexamethasone; HD-MA, high-dose methotrexate+ara-C; ICE-dex, ifosfamide+carboplatin+etoposide+dexamethasone; MP, melphalan+prednisolone; VAD, vincristine+doxorubicin+dexamethasone; VMP, bortezomib+melphalan+prednisolone; ISSWM, International Prognostic Scoring System for Waldenstrom Macroglobulinemia; Int, Intermediate; CR, complete response; PR, partial response; MR, minimal response; SD, stable disease; PD, progressive disease..

Table 3 OS and PFS of patients in the 2 groups (LPL/WM vs. non-IgM).

Abbreviations: CI, confidence interval; LPL, lymphoplasmacytic lymphoma; OS, overall survival; PFS, progression-free survival; WM, Waldenström's macroglobulinemia..