To determine the clinical and laboratory predictors of lymphoma development in primary Sjögren's syndrome (pSS). Seventy-two patients with pSS were studied. Demographic data, clinical features, serum gamma-globulin levels, autoantibodies, and HLA status were reviewed. For statistical analysis, Mann-Whitney U-test, Fisher's exact test, logistic regression analysis, Kaplan-Meier method, and log-rank tests were applied. Five patients developed a distinct non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue (MALT). A history of swollen salivary glands, lymphadenopathy, and leg ulcers predicted lymphoma development. Patients with pSS are at increased risk of lymphoma development, and those who have the above risk factors must be carefully observed.
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