Good clinical response following Ibrutinib treatment of a rare case of lymphoplasmacytic lymphoma secreting IgA kappa paraprotein: A case report

Nikolova,Dragomira; Yordanov,Alexandar; Maslarova,Alexandra; Sokolova,Liliya; Radinov,Atanas

doi:10.3892/ol.2024.14471

Good clinical response following Ibrutinib treatment of a rare case of lymphoplasmacytic lymphoma secreting IgA kappa paraprotein: A case report

Authors:
- Dragomira Nikolova
- Alexandar Yordanov
- Alexandra Maslarova
- Liliya Sokolova
- Atanas Radinov
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Affiliations: Department of Medical Genetics, Medical Faculty, Medical University - Sofia, 1431 Sofia, Bulgaria, Clinic of Hematology, University Hospital ‘St. Ivan Rilski’, 1431 Sofia, Bulgaria
Published online on: May 28, 2024 https://doi.org/10.3892/ol.2024.14471
Article Number: 338
Copyright: © Nikolova et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Lymphoplasmacytic lymphoma (LPL) is a malignant proliferation of small lymphocytes, lymphoplasmocytoid cells and plasmocytes affecting the bone marrow, lymph nodes and spleen. Its incidence is 1/100,000 and represents 8% of all lymphomas. A total of ~5% of patients with LPL may secrete non‑IgM of IgG, IgA, kappa or lambda type or be non‑secretory. In the present study, a case of a 62‑year‑old female patient who was diagnosed with non‑IgM LPL with kappa light chain monoclonal paraprotein production and normal serum immunoglobulin levels was reported. The MYD88 L265P mutation was detected by molecular genetic analysis using a sample of the bone marrow. The patient underwent initial treatment with a combination of Bendamustine‑Rituximab, and later on, Ibrutinib (a Bruton kinase inhibitor) was added to the treatment protocol. The authors' aim was to describe a case of a rare type of LPL studied and cured at the University Hospital ‘St. Ivan Rilski’, as well as to show the methods used for its diagnosis and their applicability. The difficulty in diagnosing such rare cases of LPL which are associated with marked plasmacytic differentiation and IgA paraprotein secretion resembling plasma cell neoplasia was addressed. From the other side, the characteristic features in favor of LPL diagnosis are the immunophenotype profile of plasmocytes, as well as the presence of MYD88 L265P mutation. Finally, the methods of management and treatment of this type of lymphoma were reported, highlighting the favorable effect of the treatment with Bruton TK inhibitor (Ibrutinib).

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