Abstract
We report a rare case of vanishing bile duct syndrome (VBDS) associated with diffuse large B cell lymphoma (DLBCL). VBDS is an uncommon ductopenic disorder associated with various underlying conditions, for which timely treatment is crucial to prevent poor outcomes. Our patient received six cycles of R-CHOP chemotherapy every 21 days with halved doxorubicin and vincristine doses, and prophylactic intrathecal chemotherapy. A complete response of DLBCL and normalization of liver parameters were achieved upon completion of treatment. We conclude that, when VBDS is diagnosed, one can consider to urgently reach lymphoma remission even balancing the dose reduction of hepatotoxic drugs.