A case of a 62-year-old female with Sjögren's syndrome (SjS) who developed a malignant lymphoma (ML) is reported. The patient who became blind at 32 years of age due to retinitis, began to complain of sicca syndrome (dry eye and dry mouth) and polyarthralgia at 40 years of age. A rapidly progressive subcutaneous mass was noted in the right anterior chest wall at 62 years of age. The surgically resected mass was diagnosed histologically as ML, diffuse, large cell type. Immunohistological study was done. Almost all of the lymphocytes infiltrated around ductules of a small salivary gland biopsied 6 years earlier were B-cells (MB-1; +, Mx-Pan B; +, IgG k; +). Only a small number of them were evaluated as T-cells (UCHL-1; +). The ML tumor was studied as a B-cell lymphoma which did not produce immunoglobulins (MB-1; +, Mx-Pan B; +). The patient was treated with a CHOP-B protocol and radiation without exacerbation. In recent reports of this association, helper T-cells were dominant in the lymphocytes infiltrated in the salivary gland in the early stage when ML did not develop yet. In this case, B-cells were dominant in the small salivary gland in contrast to the former reports. It is interesting that there was a difference in the character of the infiltrating lymphocytes.

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