Background & Aims: A number of immunosuppressive therapies have not been associated with clinical benefit in primary biliary cirrhosis (PBC). The use of more potent immunomodulatory therapies, however, could raise concerns over the risk of adverse effects such as the development of lymphoma. Therefore, we sought to describe the frequency, clinical spectrum, and outcomes among patients with PBC and a concomitant diagnosis of lymphoma. Methods: Patients with PBC evaluated at the Mayo Clinic between January 1, 1976-December 31, 1997 comprised the study cohort. Medical records were abstracted for demographic and clinical information including the temporal relationship between diagnoses and potential risk factors for lymphoma. Results: From a total of 2192 patients, the frequency of lymphoma was observed at 0.6% (13 cases). The majority of patients (54%) had PBC before they were diagnosed with lymphoma, whereas 23% of cases were diagnosed in conjunction with PBC. Non-Hodgkin's lymphoma (69%) was observed in most cases with disease involvement above the diaphragm (38%). Treatment in the form of chemotherapy (n = 3), radiation (n = 2), and surgery (n = 6) was provided for 11 patients. Seven (54%) patients did not have evidence for recurrent disease, whereas 3 (23%) patients died of progressive lymphoma. Conclusions: The baseline risk for lymphoma in patients with PBC is estimated at <1%. Clinical trials with potent immunosuppressive therapies for PBC should not be prohibited on the basis of concerns regarding a baseline predisposition for lymphoma in this population.
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