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Abstract

Lymphoplasmacytic Lymphoma with IgA Paraprotein: a Case Report and Literature Review by Yingying Sun, Liyan Li, Fengjuan Jiang, Hui Liu, Shan Gao, Honglei Wang, Yue Ren, Jia Song, Huaquan Wang, Zonghong Shao, Rong Fu, Lijuan Li

Background: Lymphoplasmacytic Lymphoma (LPL) with immunoglobulin (Ig)A paraprotein is rare. When plasma cells dominate, the diagnosis becomes more challenging.
Methods: We reported a case of a 71-year-old male with elevated creatinine, splenomegaly, monoclonal IgA, and MYD88 mutation. Only monoclonal plasma cells were detected first, leading to a misdiagnosis of multiple myeloma. When progressive spleen enlargement was observed, re-evaluation revealed the emergence of monoclonal lymphocytes and the diagnosis was revised to LPL.
Results: The addition of rituximab to DVD regimen led to a partial response.
Conclusions: For cases where an initial definitive diagnosis cannot be established, close follow-up is required for timely diagnosis revision and therapeutic adjustment.

DOI: 10.7754/Clin.Lab.2025.250425