title: Complete remission of paraneoplastic vanishing bile duct syndrome after the successful treatment of Hodgkin's lymphoma: a case report and review of the literature.
abstract: BACKGROUND
  Vanishing 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.
  
  
  METHODS
  We 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.
  
  
  CONCLUSIONS
  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. 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.
authors.forename: Delia
authors.initials: D
authors.lastname: Rota Scalabrini
authors.forename: Daniela
authors.initials: D
authors.lastname: Caravelli
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Carnevale Schianca
authors.forename: Lorenzo
authors.initials: L
authors.lastname: D'Ambrosio
authors.forename: Francesco
authors.initials: F
authors.lastname: Tolomeo
authors.forename: Paola
authors.initials: P
authors.lastname: Boccone
authors.forename: Antonio
authors.initials: A
authors.lastname: Manca
authors.forename: Giovanni
authors.initials: G
authors.lastname: De Rosa
authors.forename: Annamaria
authors.initials: A
authors.lastname: Nuzzo
authors.forename: Massimo
authors.initials: M
authors.lastname: Aglietta
authors.affiliation: Division of Oncology, Candiolo Cancer Institute, FPO, IRCCS, University of Torino Medical School, Candiolo, Italy. giovanni.grignani@ircc.it.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Grignani
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001649:Bile Duct Diseases / Q000150:complications / Q000628:therapy*; D005260:Female; D006689:Hodgkin Disease / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D010257:Paraneoplastic Syndromes / Q000150:complications / Q000628:therapy*; D011241:Prednisone / Q000008:administration & dosage; D012074:Remission Induction; D014580:Ursodeoxycholic Acid / Q000008:administration & dosage
chemical_list: D014580:Ursodeoxycholic Acid; D011241:Prednisone