We report a case of 55-year-old female with peripheral T-cell lymphoma (PTL) associated with Sjögren's syndrome. She was admitted to Jichi Medical School Hospital because of left axillary lymphadenopathy on August 9, 1984. Histological examination of the lymph nodes obtained by the first biopsy showed residual lymph follicles and an infiltration of immunoblasts, plasma cells, and epithelioid cells with capillary proliferation. A histological diagnosis of immunoblastic lymphadenopathy (IBL)-like lesion was suggested. The second and third lymph nodes biopsies were carried out. The histological findings did not fit any type of the Lymphoma Study Group (LSG) classification of Japan, and a diagnosis of PTL was considered. Immunohistochemical studies utilizing avidin-biotin-peroxidase complex (ABC) technique confirmed that proliferating cells predominated by peripheral T-cell with helper/inducer phenotype. Laboratory data disclosed normal level of serum LDH and no elevation of anti-virus titer of adult T-cell leukemia/lymphoma (ATLA). This case was considered to have clinical stage II B lymphoma.
The patient noticed xerostomia and foreign body sensation in the eyes several years before admission. Histological findings of minor salivary glands obtained by biopsy were compatible with the oral component of Sjögren's syndrome.
On October 11, 1984, chemotherapy was initiated with cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP) following irradiation to left axillary fields in a total dose of 40 Gy. She remained in complete remission for 12 months.
In the literature, malignant lymphoma associated with Sjögren's syndrome is mainly a monoclonal B-cell neoplasm. In this case, however, proliferating cells in the lymph nodes were polymorphous and immunohistochemical studies confirmed that they were of peripheral T-cell origin.
