[Primary hepatic diffuse large B-cell lymphoma developed in a patient with primary biliary cholangitis].

Primary hepatic lymphoma (PHL) is a lymphoproliferative disorder confined to the liver, with no evidence of lymphomatous involvement in other organs. Here, we report a case of diffuse large B-cell lymphoma (DLBCL)-type PHL in a patient with a long history of primary biliary cholangitis (PBC) and Sjögren's syndrome (SS). A 78-year-old woman presented with epigastralgia and was found to have a solitary liver tumor by contrast-enhanced computed tomography (CT). A CT scan performed 8 months earlier was unremarkable, with no extrahepatic lesions observed. The patient underwent laparoscopic left lateral segmentectomy, and the resected specimen showed histological features of DLBCL in the background of PBC-affected liver parenchyma. The patient received postoperative R-CHOP chemotherapy and has remained in complete remission for 4 years. PBC-associated PHL is extremely rare, and five of six reported cases of PBC-associated PHL cases showed a histological subtype of MALT lymphoma. In this case, immune dysregulation caused by PBC and secondary SS may have contributed to the development of DLBCL-type PHL.