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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer - Medknow
2022
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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. |
format | Online Article Text |
id | pubmed-8982943 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Wolters Kluwer - Medknow |
record_format | MEDLINE/PubMed |
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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