Abstract
BackgroundVanishing bile duct syndrome has been associated with different pathologic conditions (adverse drug reactions, autoimmune diseases, graft versus host disease, and cancer). Though its causes are unknown, an immune-related pathogenesis is the most likely one. Vanishing bile duct syndrome can evolve to hepatic failure and, eventually, to death. The treatment is uncertain, but it needs the resolution of the underlying pathologic condition.Case presentationWe describe the association of Hodgkin’s lymphoma with a syndrome characterized by cholestasis, aminotransferase elevation and an histological picture of bile duct loss. All other causes of hepatic function impairment were excluded (in particular, drugs, viral and autoimmune related diseases) eventually leading to the diagnosis of vanishing bile duct syndrome. Despite the fact that the dysfunction is not caused by hepatic Hodgkin’s lymphoma involvement, liver impairment can limit the optimal therapy of Hodgkin’s lymphoma. A treatment consisting of ursodeoxycholic acid, prednisone, and full dose chemotherapy restored hepatic function and achieved complete and long-lasting remission of Hodgkin’s lymphoma.ConclusionWe reviewed all case reports showing that vanishing bile duct syndrome is a dismal paraneoplastic syndrome being fatal in a high proportion of patients if not adequately treated. Indeed, this syndrome requires both an early recognition and an appropriate aggressive treatment consisting of full dose upfront chemotherapy which is the only way to achieve a resolution of the vanishing bile duct syndrome. Delayed or reduced intensity treatments unfavorably correlate with survival.
Highlights
Vanishing bile duct syndrome has been associated with different pathologic conditions
We reviewed all case reports showing that vanishing bile duct syndrome is a dismal paraneoplastic syndrome being fatal in a high proportion of patients if not adequately treated
This syndrome requires both an early recognition and an appropriate aggressive treatment consisting of full dose upfront chemotherapy which is the only way to achieve a resolution of the vanishing bile duct syndrome
Summary
Several conditions may cause jaundice in HL. Biliary obstruction secondary to lymph nodes enlargement, hemolysis, hepatotropic and other viruses (e.g. CMV) are among the most common causes. Only 1 out of 8 (12%) patients receiving an upfront reduced chemotherapy overcame the vanishing bile duct, compared to 51% (17/33) of patients treated with upfront full dose chemotherapy (see Table 1) In these patients the efficacious treatment of the lymphoma preceded the hepatic improvement and, the cure. This case report demonstrates that in the presence of a histological diagnosis of HL associated with signs of cholestasis and liver function impairment without gross liver involvement or biliary tract dilatation, the prompt treatment of the underlying disease in association with high doses of UDCA is crucial. All authors revised the manuscript and approved the final version for submission
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