Lab Med Online LMO Laboratory Medicine Online 2093-6338 The Korean Society for Laboratory Medicine 10.3343/lmo.2019.9.4.263 Case Report A Case of Lymphoplasmacytic Lymphoma/Waldenström's Macroglobulinemia with IgM-κ and IgA-λ Biclonal Gammopathy Shin Woo Yong M.D. https://orcid.org/0000-0001-7854-3011 Bang Hae In M.D. Kim Jieun M.D. Park Rojin M.D. Shin Jeong Won M.D. Choi Tae Youn M.D. Department of Laboratory Medicine, Soonchunhyang University Seoul Hospital, Seoul, Korea. Corresponding author: Hae In Bang, M.D. Department of Laboratory Medicine, Soonchunhyang University Seoul Hospital, 59 Daesagwan-ro, Yongsan-gu, Seoul 04401, Korea. Tel: +82-2-709-9430, Fax: +82-2-710-3088, genuine43@schmc.ac.kr 10 2019 01 10 2019 9 4 263 268 04 01 2019 10 05 2019 22 05 2019 © 2019, Laboratory Medicine Online 2019 Laboratory Medicine Online 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.

Lymphoplasmacytic lymphoma (LPL) is a low-grade B-cell neoplasm, composed of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow and sometimes lymph nodes or spleen. LPL with bone marrow involvement and an IgM monoclonal gammopathy of any concentration is designated as Waldenström macroglobulinemia (WM). LPL associated with non-IgM monoclonal gammopathy or biclonal gammopathy is rarely observed. LPL diagnosis was based on clinical, morphological, and immunophenotypic findings. Recently, the test for L265P mutation of the myeloid differentiation factor 88 (MYD88) gene has been helpful in the diagnosis of LPL. Here, we reported the first case of LPL/WM with IgM-κ/IgA-λ biclonal gammopathy in Korea.

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Two monoclonal peaks, IgA-λ and IgM-κ types, are observed in gamma-region. IgA-λ type monoclonal peak is dominant. M protein was 3.04 g/dL. Bone marrow observations. (A) Microscopic observation of bone marrow (×1,000). Hypercellular marrow with markedly increased lymphocytes. Mixture of small lymphocytes, plasma cells, and plasmacytic lymphocytes formed clusters. Mast cells increased in number. (B) Bone marrow biopsy shows interstitial infiltration of plasmacytic lymphocytes (×400). (C) Immunophenotypic investigation revealed that neoplastic cells express CD19+ and CD56− and exhibit cytoplasmic lambda restriction. The Neoplastic cell population shows cells with a range of CD138 expression, from CD138 dim plasmacytic lymphocytes to CD138 bright mature plasma cells. (D) Next-generation sequencing (NGS) revealed <italic>MYD88</italic> L265P mutation in 4.36% of bone marrow cells (depth: 436).