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IgG4-related disease (IgG4-RD) which is characterized by an autoimmune abnormality and elevated serum IgG4 level often presents as swelling, nodules, and the thickening of multiple organs. It typically occurs in middle-aged to elderly patients, but its pathogenesis remains unclear. Lymphadenopathy is frequently seen in IgG4-RD, which clinically requires a differential diagnosis from malignant lymphoma. We herein report a case of malignant lymphoma with IgG4-RD arising in a very elderly man.An 85-year old man with a 6-year history of auto