Introduction: Sjögren's syndrome is a chronic autoimmune disorder carrying the risk of the development of non-Hodgkin's lymphoma, most frequently marginal zone lymphoma.
Case outline: A 66-year-old male patient with Sjögren's syndrome, after a year of the disease, developed a nodal marginal zone lymphoma with lymphoma cells in peripheral blood which had the following immunophenotype: CD19, CD20, CD22, CD19/kappa, CD79b+. After six cycles of chemotherapy according to CHOP protocol (cyclophosphamide, doxorubicin, vincristine and prednisone) disease remission was achieved lasting four months, followed by enlargement of lymph nodes in all areas (generalized lymphadenopathy), splenomegaly and enlargement of the right parotid gland. Bone marrow biopsy and histology confirmed lymphoma of the same morphologic and immunohistochemic profile. Biopsy of a very enlarged hard right parotid gland, by using histology and immunohistochemistry, showed lymphoid tumour tissue with blast appearance and a number of nucleoli corresponding to centroblasts and less to immunoblasts. Immunophenotypes of these cells were as follows: CD79alfa+, CD20+, CD3-, bcl-2-; proliferative activity measured with KI-67 was high rating 60%. Histology and immunohistochemistry showed the co-existence of a diffuse large B cell lymphoma with marginal zone lymphoma. In spite of aggressive chemotherapy treatment according to protocol ESHAP (Vepesid 200 mg i.v. on 1st and 2nd day and 100 mg on 3rd, 4th and 5th day; Cisplatin 20-20-10 mg on 1st to 4th day) the disease showed a progressive course.
Conclusion: In patients with Sjögren's syndrome, the possibility of lymphoma should be kept in mind and in suspected cases timely diagnostic and therapeutic measures should be undertaken.