title: Vanishing bile duct syndrome after allogeneic bone marrow transplantation: is it the end of the road?
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.
authors.affiliation: Ankara University Medical School, Department of Hematology, Ankara, Turkey.
authors.forename: Şule Mine
authors.initials: ŞM
authors.lastname: Bakanay
authors.forename: Aynur Uğur
authors.initials: AU
authors.lastname: Bilgin
authors.forename: Mehmet
authors.initials: M
authors.lastname: Bektaş
authors.forename: Ramazan
authors.initials: R
authors.lastname: Idilman
authors.forename: Esra
authors.initials: E
authors.lastname: Erden
authors.forename: Mutlu
authors.initials: M
authors.lastname: Arat
authors.forename: Önder
authors.initials: Ö
authors.lastname: Arslan
mesh_terms: D001649:Bile Duct Diseases / Q000209:etiology* / Q000473:pathology*; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D016026:Bone Marrow Transplantation / Q000009:adverse effects; D058625:End Stage Liver Disease / Q000209:etiology / Q000473:pathology; D017809:Fatal Outcome; D006086:Graft vs Host Disease / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000628:therapy*; D008297:Male; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents