Lab Med Online.  2019 Oct;9(4):263-268. 10.3343/lmo.2019.9.4.263.

A Case of Lymphoplasmacytic Lymphoma/Waldenström's Macroglobulinemia with IgM-κ and IgA-λ Biclonal Gammopathy

• Shin WY
• Bang HI
• Kim J
• Park R
• Shin JW
• Choi TY

• KMID: 2458690
• DOI: http://doi.org/10.3343/lmo.2019.9.4.263

Abstract

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.

Keyword

Lymphoplasmacytic lymphoma; Waldenström's macroglobulinemia; Myeloid differentiation factor 88; Multiple myeloma; Biclonal paraproteinemia

MeSH Terms

B-Lymphocytes
Bone Marrow
Diagnosis
Immunoglobulin M
Korea
Lymph Nodes
Lymphocytes
Lymphoma
Multiple Myeloma
Myeloid Differentiation Factor 88
Paraproteinemias
Plasma Cells
Spleen
Waldenstrom Macroglobulinemia*
Immunoglobulin M
Myeloid Differentiation Factor 88

Figure

• 

  Fig. 1 Immunofixation electrophoresis (serum). 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.

• 

  Fig. 2 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 MYD88 L265P mutation in 4.36% of bone marrow cells (depth: 436).

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