Marginal zone lymphoma of mucosa associated lymphoid tissue-lymphoma of the lacrimal gland in a young patient with Klinefelter syndrome: a case report

Abstract Background Klinefelter syndrome is considered one of the most common sex chromosome disorders affecting males. The presence of an extra X chromosome can alter the tendency to develop various cancers, including lymphomas. Lacrimal gland lymphoma is a disease of the elderly, with a median age...

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Main Authors: Askar K. Alshaibani, Reem R. Alanazi, Bedour Akif Aleid, Saad M. Alosaimi
Format: Article
Language:English
Published: BMC 2024-12-01
Series:Journal of Medical Case Reports
Subjects:
Online Access:https://doi.org/10.1186/s13256-024-04996-0
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author Askar K. Alshaibani
Reem R. Alanazi
Bedour Akif Aleid
Saad M. Alosaimi
author_facet Askar K. Alshaibani
Reem R. Alanazi
Bedour Akif Aleid
Saad M. Alosaimi
author_sort Askar K. Alshaibani
collection DOAJ
description Abstract Background Klinefelter syndrome is considered one of the most common sex chromosome disorders affecting males. The presence of an extra X chromosome can alter the tendency to develop various cancers, including lymphomas. Lacrimal gland lymphoma is a disease of the elderly, with a median age of presentation of 70 years. Case presentation In this article, we report a case of a 35-year-old Arabian Saudi male with a known case of Klinefelter syndrome who presented to oculoplastic clinic complaining of progressively growing superior-temporal mass in the left eye. After evaluation and imaging, an incisional biopsy from the lacrimal gland mass was obtained and histopathological evaluation showed atypical lymphoproliferative infiltrate consistent with extranodal marginal zone lymphoma of mucosa associated lymphoid tissue. The mainstay treatment was external beam radiotherapy, which showed significant improvement in the case. Conclusion This is considered the first reported case of lacrimal gland lymphoma in young patient with Klinefelter syndrome, which increases the association between Klinefelter syndrome and lymphomas.
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spelling doaj-art-ce97a787cc4e4b7189c3f9a429b7565f2025-08-20T02:43:36ZengBMCJournal of Medical Case Reports1752-19472024-12-011811510.1186/s13256-024-04996-0Marginal zone lymphoma of mucosa associated lymphoid tissue-lymphoma of the lacrimal gland in a young patient with Klinefelter syndrome: a case reportAskar K. Alshaibani0Reem R. Alanazi1Bedour Akif Aleid2Saad M. Alosaimi3Dhahran Eye Specialist HospitalDepartment of Ophthalmology, College of Medicine, King Fahad Hospital of the University, Imam Abdulrahman Bin Faisal UniversityDhahran Eye Specialist HospitalMinistry of HealthAbstract Background Klinefelter syndrome is considered one of the most common sex chromosome disorders affecting males. The presence of an extra X chromosome can alter the tendency to develop various cancers, including lymphomas. Lacrimal gland lymphoma is a disease of the elderly, with a median age of presentation of 70 years. Case presentation In this article, we report a case of a 35-year-old Arabian Saudi male with a known case of Klinefelter syndrome who presented to oculoplastic clinic complaining of progressively growing superior-temporal mass in the left eye. After evaluation and imaging, an incisional biopsy from the lacrimal gland mass was obtained and histopathological evaluation showed atypical lymphoproliferative infiltrate consistent with extranodal marginal zone lymphoma of mucosa associated lymphoid tissue. The mainstay treatment was external beam radiotherapy, which showed significant improvement in the case. Conclusion This is considered the first reported case of lacrimal gland lymphoma in young patient with Klinefelter syndrome, which increases the association between Klinefelter syndrome and lymphomas.https://doi.org/10.1186/s13256-024-04996-0Lacrimal glandLymphomaKlinefelter syndrome
spellingShingle Askar K. Alshaibani
Reem R. Alanazi
Bedour Akif Aleid
Saad M. Alosaimi
Marginal zone lymphoma of mucosa associated lymphoid tissue-lymphoma of the lacrimal gland in a young patient with Klinefelter syndrome: a case report
Journal of Medical Case Reports
Lacrimal gland
Lymphoma
Klinefelter syndrome
title Marginal zone lymphoma of mucosa associated lymphoid tissue-lymphoma of the lacrimal gland in a young patient with Klinefelter syndrome: a case report
title_full Marginal zone lymphoma of mucosa associated lymphoid tissue-lymphoma of the lacrimal gland in a young patient with Klinefelter syndrome: a case report
title_fullStr Marginal zone lymphoma of mucosa associated lymphoid tissue-lymphoma of the lacrimal gland in a young patient with Klinefelter syndrome: a case report
title_full_unstemmed Marginal zone lymphoma of mucosa associated lymphoid tissue-lymphoma of the lacrimal gland in a young patient with Klinefelter syndrome: a case report
title_short Marginal zone lymphoma of mucosa associated lymphoid tissue-lymphoma of the lacrimal gland in a young patient with Klinefelter syndrome: a case report
title_sort marginal zone lymphoma of mucosa associated lymphoid tissue lymphoma of the lacrimal gland in a young patient with klinefelter syndrome a case report
topic Lacrimal gland
Lymphoma
Klinefelter syndrome
url https://doi.org/10.1186/s13256-024-04996-0
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AT bedourakifaleid marginalzonelymphomaofmucosaassociatedlymphoidtissuelymphomaofthelacrimalglandinayoungpatientwithklinefeltersyndromeacasereport
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