title: Hodgkin lymphoma-related vanishing bile duct syndrome and idiopathic cholestasis: statistical analysis of all published cases and literature review.
abstract: BACKGROUND
  Hodgkin lymphoma (HL)-related vanishing bile duct syndrome (VBDS) and idiopathic cholestasis (IC) are rare conditions that often lead to liver failure and death. The available literature consists primarily of case reports, resulting in little clarity as to the clinical course and ideal treatment for this disease.
  
  
  METHODS
  We performed a literature search from which we identified all published cases of HL-related VBDS or IC, and created a database of detailed presentation, treatment, and outcome information for all patients. Patient and disease factors were analyzed for an association with overall survival and liver failure-free survival. A case presentation introduces this analysis.
  
  
  RESULTS
  Thirty-seven cases of HL-related VBDS/IC were identified. Median follow-up was 7 months; 1-year OS and liver failure-free survival (LFFS) are 43% and 41%, respectively. Sixty-five percent of the patients died while 30% were alive with normal or near-normal stable liver function and no evidence of recurrent HL at last evaluation. Of the 20 patients without residual HL following therapy, 12 (60%) achieved liver failure-free survival. On univariate analysis, factors significantly associated with improved liver failure-free survival were stage I/II HL (p=0.02), a complete response of HL (p=0.0002), and delivery of radiotherapy (pB0.0001). Two patients received chemotherapy without radiation and survived with recovery of liver function.
  
  
  CONCLUSIONS
  HL-related VBDS/IC is potentially reversible and not uniformly fatal, with 30% of presenting patients demonstrating good lymphoma and liver outcomes after definitive therapy for HL. As a complete response of HL provides the only possibility of recovering liver function, patients with this disease should proceed to definitive treatment of HL as soon as feasible.
authors: Department of Radiation Oncology, University of Colorado, Comprehensive Cancer Center, Aurora, CO 80045-0510, USA. ari.ballonoff@uchsc.edu
authors: Ari
authors: A
authors: Ballonoff
authors: Brian
authors: B
authors: Kavanagh
authors: Russell
authors: R
authors: Nash
authors: Harry
authors: H
authors: Drabkin
authors: James
authors: J
authors: Trotter
authors: Luciano
authors: L
authors: Costa
authors: Rachel
authors: R
authors: Rabinovitch
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001649:Bile Duct Diseases / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality; D005260:Female; D006689:Hodgkin Disease / Q000150:complications* / Q000401:mortality; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D013577:Syndrome