This 53 year-old female had been admitted to our hospital in 1975 with the complaints of general malaise, hoarseness and facial edema. She was diagnosed of Hashimoto's thyroidits, chronic active hepatitis and Sjögren's syndrome by biopsies of the thyroid glands, the liver and the salivary glands and was administered prednisolone, azathioprine and thyroid hormone.
In 1981, she was admitted to our hospital for the second time with the complaint of left upper quadrant pain. The X-ray examination of the upper gastrointestinal tract and gastric endoscopy showed multiple ulcers with submucosal tumor at the gastric antrum. The biopsy specimen of the gastric mucosa suggested the diagnosis of malignant lymphoma of the stomach. However, subtotal gastrectomy was performed and surgical specimen demonstrated diffuse infiltration of plasma cells with pleomorphism. Immunohistochemical staining revealed in_??_ra cytoplasmic immunoglobulin, mainly IgM kappa and she was diagnosed of malignant extramedullary gastric plasmacytoma. Liver biopsy performed simultaneously showed characteristic changes of the primary biliary cirrhosis.
We believe that this case is the first case of Sjögren's syndrome associated with extramedullary plasmacytoma of the stomach, Hashimoto's thyroiditis and the primary biliary cirrhosis.