J Korean Ophthalmol Soc.  2016 Jul;57(7):1159-1164. 10.3341/jkos.2016.57.7.1159.

A Case of Lacrimal Gland MALT Lymphoma in a Patient with Primary Sjögren's Syndrome

• Kim HW
• Sung YM
• Baik JS
• Yang SW

• KMID: 2317580
• DOI: http://doi.org/10.3341/jkos.2016.57.7.1159

Abstract

PURPOSE
To report a case of lacrimal gland mucosa-associated lymphoid tissue (MALT) lymphoma in a patient with primary Sjögren's syndrome and Behcet's disease.
CASE SUMMARY
A 49-year-old female patient with primary Sjögren's syndrome and Behcet's disease presented with a one-year history of painless upper and lower eyelid swelling in her right eye. Lacrimal gland incisional biopsy was performed, and the patient was diagnosed with malignant lymphoma (extranodal marginal zone B cell lymphoma of MALT). No distant metastases were detected on whole-body computed tomography or positron emission tomography, and the patient was treated with Rituximab, Cyclophosphamide, Vincristine, Prednisone (R-CVP) regimen chemotherapy. After 8 consecutive chemotherapy cycles, her eyelids appeared normal externally, and partial regression was found radiologically.
CONCLUSIONS
The possibility of MALT lymphoma should be considered as a differential diagnosis if patients with autoimmune diseases such as primary Sjögren's syndrome show eyelid swelling or palpable mass.

Keyword

Autoimmune disease; Behcet's disease; Lymphoma of lacrimal gland; Mucosa-associated lymphoid tissue (MALT) lymphoma; Sjögren's syndrome

MeSH Terms

Autoimmune Diseases
Biopsy
Cyclophosphamide
Diagnosis, Differential
Drug Therapy
Eyelids
Female
Humans
Lacrimal Apparatus*
Lymphoid Tissue
Lymphoma
Lymphoma, B-Cell, Marginal Zone*
Middle Aged
Neoplasm Metastasis
Positron-Emission Tomography
Prednisone
Rituximab
Vincristine
Cyclophosphamide
Prednisone
Rituximab
Vincristine

Figure

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  Figure 1. External photograph of a 49-year-old female patient with lacrimal gland mucosa-associated lymphoid tissue lymphoma. (A) Before chemotherapy, swelling of upper and lower eyelids was prominent in the right eye. (B) One month after eight cycles of chemotherapy, the right eyelid appears nearly normal.

• 

  Figure 2. Orbital T1-weighted magnetic resonance images showing enhancing enlargement of the right lacrimal gland. (A, B) Before chemotherapy, a diffusely enlarged right lacrimal gland is noted. (C, D) One month after eight cycles of chemotherapy, the right lacrimal gland enhancement reduced dramatically.

Reference

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