Abstract
It is recognized that autoimmune diseases such as primary Sjogren's syndrome and rheumatoid arthritis have an increased risk of developing lymphoma, particularly non-Hodgkin's lymphoma (NHL). The progression of primary biliary cholangitis (PBC), a chronic autoimmune liver disease, to lymphoma on this basis has rarely been reported. This article describes a 67-year-old female patient who developed diffuse large B-cell lymphoma (DLBCL) one year after the diagnosis of PBC. She had experienced a transient improvement after receiving treatment but died as a result of infections and other related problems triggered by further immune suppression after chemotherapy. We also reviewed the relevant literature to assess the correlation between PBC and lymphoma risk.