Vanishing bile duct syndrome after allogeneic bone marrow transplantation: is it the end of the road?

Turk J Gastroenterol. 2013;24(4):359-62. doi: 10.4318/tjg.2013.0604.

Abstract

In this paper, we report the case of a 19-year-old male patient who presented with lymphoblastic phase of chronic myeloid leukemia and received an allogeneic bone marrow transplant from his cousin. The patient experienced severe, steroid-refractory acute graft versus-host disease of skin, gastrointestinal tract and liver that required further immunosuppression. However, hepatic graft-versus-host disease was complicated with vanishing bile duct syndrome, characterized by progressive destruction of small intrahepatic bile ducts, which was refractory to all available therapies and eventually led to end-stage liver disease. The pathogenesis and treatment of graft-versus-host disease after allogeneic hematopoietic cell transplantation is discussed with an emphasis on liver transplantation for intractable hepatic graft-versus-host disease.

Publication types

  • Case Reports

MeSH terms

  • Bile Duct Diseases / etiology*
  • Bile Duct Diseases / pathology*
  • Bile Ducts, Intrahepatic / pathology*
  • Bone Marrow Transplantation / adverse effects
  • End Stage Liver Disease / etiology
  • End Stage Liver Disease / pathology
  • Fatal Outcome
  • Graft vs Host Disease / drug therapy
  • Graft vs Host Disease / etiology
  • Graft vs Host Disease / pathology*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive / therapy*
  • Male
  • Young Adult

Substances

  • Immunosuppressive Agents