Immunoglobulin G4 Positive Mucosa-associated Lymphoid Tissue Lymphoma of the Lacrimal Gland

PURPOSE Mucosa-associated lymphoid tissue lymphoma recently has been defined as a distinct subtype of non-Hodgkin's lymphoma with characteristic clinicopathologic features. A 37-year-old woman with systemic lupus erythematosus and unilateral mucosa-associated lymphoid tissue lymphoma of the lacrimal gland is described. METHODS The tumor was totally excised by lateral orbitotomy. Immunohistochemical studies were performed with UCHL-1, CD20 (L26), leukocyte common antigen, cytokeratin (CAM5), and kappa and lambda light chain antibodies. RESULTS The tumor was composed of centrocyte-like lymphocytes, cells with plasmacytoid features, and lymphoepithelial lesions. Most of the cells expressed the CD20 protein and were positive for immunoglobulin kappa light chain. The patient received no supplemental therapy. No systemic dissemination or local recurrence occurred during a follow-up of 26 months. CONCLUSION The features of this case support the association between systemic diseases and the subsequent development of mucosa-associated lymphoid tissue lymphoma.