Marginal Zone Lymphoma of Mucosa Associated Lymphoid Tissue-lymphoma of the Lacrimal Gland in a Young Patient with Klinefelter Syndrome: a Case Report

Overview

Journal J Med Case Rep

Publisher Biomed Central

Specialty General Medicine

Date 2024 Dec 26

PMID 39726031

Authors

Askar K Alshaibani

Reem R Alanazi

Bedour Akif Aleid

Saad M Alosaimi

Affiliations

Soon will be listed here.

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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