Splenic marginal zone B-cell lymphoma associated with primary Sjögren’s syndrome

Abstract
We describe a 67-year-old woman with primary Sjögren’s syndrome who developed a splenic marginal zone B-cell lymphoma, and complained of a dry mouth and Raynaud’s phenomenon. She had splenomegaly, swollen lymph nodes and monoclonal IgM-κ cryoglobulin. After splenectomy, Raynaud’s phenomenon had improved with the decrease of cyroglobulin. This is the first case of primary Sjögren’s syndrome with splenic marginal zone B-cell lymphoma to be reported.