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Ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-G4-related ophthalmic disease: A 4-year delay in diagnosis

Ocular adnexal marginal zone B cell lymphomas (MZBLs) make up the majority of lymphomas arising from the ocular adnexa. Immunoglobulin-G4 (IgG4)-related disease is a recently proposed entity with several unique clinicopathological features, such as enlargement of affected organs, elevated serum IgG4...

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Autores principales: Sabt, Buthaina I., Al Yahyai, Majda, Al-Mujaini, Arwa A., Al-Mujaini, Abdullah S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8982943/
https://www.ncbi.nlm.nih.gov/pubmed/35391806
http://dx.doi.org/10.4103/1319-4534.337858
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author Sabt, Buthaina I.
Al Yahyai, Majda
Al-Mujaini, Arwa A.
Al-Mujaini, Abdullah S.
author_facet Sabt, Buthaina I.
Al Yahyai, Majda
Al-Mujaini, Arwa A.
Al-Mujaini, Abdullah S.
author_sort Sabt, Buthaina I.
collection PubMed
description Ocular adnexal marginal zone B cell lymphomas (MZBLs) make up the majority of lymphomas arising from the ocular adnexa. Immunoglobulin-G4 (IgG4)-related disease is a recently proposed entity with several unique clinicopathological features, such as enlargement of affected organs, elevated serum IgG4 level, and infiltration with IgG4-positive plasma cells. Ocular adnexal MZBLs are reported to arise in IgG4-related sclerosing dacryoadenitis, indicating a possible link between the two conditions. Here, we describe a 37-year-old Omani male who presented with right periorbital swelling and proptosis 4 years before presentation. He was diagnosed to have right orbital pseudotumor and exhibited good response to steroid therapy. However, 4 years later, rapid swelling of the right orbital mass was observed. The patient underwent lacrimal gland biopsy. Although the histology was consistent with IgG4-related disease, the infiltrating large atypical lymphoid cells showed that immunoglobulin light-chain restriction and dense lymphoplasmacytic infiltrate involving the soft tissue were seen. Consequently, he was diagnosed with extranodal marginal zone lymphoma with abundant IgG4-positive cells of the right lacrimal gland.
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spelling pubmed-89829432022-04-06 Ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-G4-related ophthalmic disease: A 4-year delay in diagnosis Sabt, Buthaina I. Al Yahyai, Majda Al-Mujaini, Arwa A. Al-Mujaini, Abdullah S. Saudi J Ophthalmol Case Report Ocular adnexal marginal zone B cell lymphomas (MZBLs) make up the majority of lymphomas arising from the ocular adnexa. Immunoglobulin-G4 (IgG4)-related disease is a recently proposed entity with several unique clinicopathological features, such as enlargement of affected organs, elevated serum IgG4 level, and infiltration with IgG4-positive plasma cells. Ocular adnexal MZBLs are reported to arise in IgG4-related sclerosing dacryoadenitis, indicating a possible link between the two conditions. Here, we describe a 37-year-old Omani male who presented with right periorbital swelling and proptosis 4 years before presentation. He was diagnosed to have right orbital pseudotumor and exhibited good response to steroid therapy. However, 4 years later, rapid swelling of the right orbital mass was observed. The patient underwent lacrimal gland biopsy. Although the histology was consistent with IgG4-related disease, the infiltrating large atypical lymphoid cells showed that immunoglobulin light-chain restriction and dense lymphoplasmacytic infiltrate involving the soft tissue were seen. Consequently, he was diagnosed with extranodal marginal zone lymphoma with abundant IgG4-positive cells of the right lacrimal gland. Wolters Kluwer - Medknow 2022-02-18 /pmc/articles/PMC8982943/ /pubmed/35391806 http://dx.doi.org/10.4103/1319-4534.337858 Text en Copyright: © 2022 Saudi Journal of Ophthalmology https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Sabt, Buthaina I.
Al Yahyai, Majda
Al-Mujaini, Arwa A.
Al-Mujaini, Abdullah S.
Ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-G4-related ophthalmic disease: A 4-year delay in diagnosis
title Ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-G4-related ophthalmic disease: A 4-year delay in diagnosis
title_full Ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-G4-related ophthalmic disease: A 4-year delay in diagnosis
title_fullStr Ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-G4-related ophthalmic disease: A 4-year delay in diagnosis
title_full_unstemmed Ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-G4-related ophthalmic disease: A 4-year delay in diagnosis
title_short Ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-G4-related ophthalmic disease: A 4-year delay in diagnosis
title_sort ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-g4-related ophthalmic disease: a 4-year delay in diagnosis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8982943/
https://www.ncbi.nlm.nih.gov/pubmed/35391806
http://dx.doi.org/10.4103/1319-4534.337858
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