Treatment of hepatic amyloid light-chain amyloidosis with bortezomib and dexamethasone in a liver transplant patient

Abstract

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 83days after the LT, with normal liver and renal function, and no clinical evidence of recurrent disease was observed at the latest follow up (22months post-LT). © 2015 John Wiley