Premium
Treatment of hepatic amyloid light‐chain amyloidosis with bortezomib and dexamethasone in a liver transplant patient
Author(s) -
Nakano Ryosuke,
Ohira Masahiro,
Ide Kentaro,
Ishiyama Kohei,
Kobayashi Tsuyoshi,
Tahara Hiroyuki,
Tashiro Hirotaka,
Kuroda Yoshiaki,
Ichinohe Tatsuo,
Arihiro Koji,
Chayama Kazuaki,
Ohdan Hideki
Publication year - 2015
Publication title -
hepatology research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.123
H-Index - 75
eISSN - 1872-034X
pISSN - 1386-6346
DOI - 10.1111/hepr.12462
Subject(s) - amyloidosis , medicine , al amyloidosis , bortezomib , amyloid (mycology) , dexamethasone , hemodialysis , liver transplantation , chemotherapy , pathology , gastroenterology , transplantation , multiple myeloma , immunoglobulin light chain , immunology , antibody
Hepatic amyloid light‐chain ( AL ) amyloidosis is characterized by abnormal deposition of amyloid fibrils in the liver. As this precursor protein is produced by a proliferative plasma cell clone in the bone marrow, liver transplantation ( LT ) does not affect the disease's progression. Here, we describe the successful treatment using bortezomib‐ and dexamethasone‐based chemotherapy, following LT , of hepatic AL amyloidosis in a 65‐year‐old woman with progressive liver failure. The patient presented with progressive hepatic dysfunction accompanied by hepatorenal syndrome requiring hemodialysis, and living donor LT was successfully performed. Histology revealed amyloid deposits in the liver and stomach, and serum immunofixation revealed AL amyloidosis (κ‐type). The patient began chemotherapy on day 45 after the LT , and remission was achieved after one course. She was subsequently discharged 83 days after the LT , with normal liver and renal function, and no clinical evidence of recurrent disease was observed at the latest follow up (22 months post‐ LT ).