A 33-year-old woman with systemic lupus erythematosus had bilateral eyelid swelling. On examination, bilateral lacrimal gland mass were palpable. The morphologic and the immunohistochemical features were supportive of the diagnosis of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue-type (MALT lymphoma). Further systemic evaluation revealed she had clinical stage IV disease with bone marrow involvement. She underwent local radiotherapy and several cycles of chemotherapy. However, the patient died of sepsis with the disease after 48 months. Systemically disseminated MALT lymphoma presenting initially in the lacrimal gland of a patient with systemic lupus erythematosus is rare, and the prognosis is poor.
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