Severe isolated acute hepatic graft-versus-host disease with vanishing bile duct syndrome

A 28-year-old man with chronic myelogenous leukaemia in blastic transformation underwent allogeneic bone marrow transplantation from his HLA-identical brother. Severe, progressive cholestatic jaundice developed from day 25 and did not respond to repeated therapy with high-dose methylprednisolone. In addition to marked cholestasis, both liver biopsy (day 69) and autopsy (day 134) findings revealed total disappearance of interlobular bile ducts in all of the portal areas, although extrahepatic manifestations of GVHD were minimal. Isolated acute vanishing bile duct syndrome can occur as the most severe form of acute hepatic GVHD.