Sjögren’s syndrome is a chronic autoimmune disease characterized by lymphoproliferative infiltration of exocrine glands. Infiltration leads to deterioration of gland and alteration of its function such as loss of tears and decreased salivary excretion. Course of the disease is characterized by either local autoimmune process or systemic disease affecting many internal organs. Regarding the fact that Sjögren’s syndrome is mostly associated with malignant transformation to lymphoproliferative disease, it is considered as a borderline disease between autoimmunity and malignant lymphoproliferation. The results of several studies evaluating risk factors for malignant transformation are inconsistent. The enlargement of parotid gland, lymphadenopathy, palpable purpura, cutaneous vasculitis and neuropathy belong to the most frequently referred clinical risk factors. Lymphocytopenia, decline in CD4 positive lymphocytes, hypogammaglobulinemia, monoclonal gammapathy, cryoglobulinemia and low levels of complement C3 and C4 represent the most frequently referred laboratory risk factors. Presented case report describes most of abovementioned risk factors in a patient who demonstrated transformation from primary Sjögren’s syndrome to diffuse large B-cell non-Hodgkin lymphoma with kappa positive plasmocellular differentiation.

        Key words: Sjögren’s syndrome, lymphoma, risk factors for malignant transformation