# MYD88‐mutated Lymphoplasmacytic Lymphoma With Monoclonal Immunoglobulin G: A Case Report

**Authors:** Morten Yung Isaksen, Olav Karsten Vintermyr, Håkon Reikvam

PMC · DOI: 10.1002/jha2.70027 · EJHaem · 2025-03-27

## TL;DR

A rare case of lymphoplasmacytic lymphoma secreting IgG instead of IgM is reported, highlighting the importance of considering this diagnosis even without typical IgM markers.

## Contribution

This case report expands the understanding of lymphoplasmacytic lymphoma by presenting an atypical IgG-secreting variant.

## Key findings

- The patient had LPL secreting monoclonal IgG instead of the usual IgM.
- The patient was treated with bortezomib, dexamethasone, and rituximab due to disease progression.
- This case emphasizes the need to consider LPL in differential diagnosis even without IgM.

## Abstract

Lymphoplasmacytic lymphomas (LPL) are usually associated with serum monoclonal immunoglobulin M (IgM). Nevertheless, in some cases, these cells may secrete IgA or IgG monoclonal proteins or remain non‐secretory. We report a case from a patient with LPL‐secreting IgG who developed anaemia and splenomegaly during the disease course that necessitated treatment with bortezomib, dexamethasone, and rituximab. The case illustrates the need for clinicians and pathologists to consider LPLs as a differential diagnosis also without a serum monoclonal IgM.

Clinical Trial Registration: The authors have confirmed clinical trial registration is not needed for this submission.

## Linked entities

- **Proteins:** CD40LG (CD40 ligand), IGG (Immunoglobulin G level)
- **Chemicals:** bortezomib (PubChem CID 387447), dexamethasone (PubChem CID 5743)
- **Diseases:** lymphoplasmacytic lymphoma (MONDO:0000432)

## Full-text entities

- **Genes:** MYD88 (MYD88 innate immune signal transduction adaptor) [NCBI Gene 4615] {aka IMD68, MYD88D, WM1}, CD79A (CD79a molecule) [NCBI Gene 973] {aka IGA, IGAlpha, MB-1, MB1}
- **Diseases:** splenomegaly (MESH:D013163), anaemia (MESH:D000743), LPLs (MESH:C535904), LPL (MESH:D008223)
- **Chemicals:** Monoclonal Immunoglobulin G (-), dexamethasone (MESH:D003907), bortezomib (MESH:D000069286), rituximab (MESH:D000069283)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11948671/full.md

## References

10 references — full list in the complete paper: https://tomesphere.com/paper/PMC11948671/full.md

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Source: https://tomesphere.com/paper/PMC11948671