Treatment of hepatic amyloid light‐chain amyloidosis with bortezomib and dexamethasone in a liver transplant patient |
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Authors: | Ryosuke Nakano Masahiro Ohira Kentaro Ide Kohei Ishiyama Tsuyoshi Kobayashi Hiroyuki Tahara Hirotaka Tashiro Yoshiaki Kuroda Tatsuo Ichinohe Koji Arihiro Kazuaki Chayama Hideki Ohdan |
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Affiliation: | 1. Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan;2. Department of Hematology and Oncology, Research Center for Radiation Casualty Medicine, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan;3. Department of Pathology, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan;4. Department of Medicine and Molecular Science, Division of Frontier Medical Science, Programs for Biomedical Research, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan |
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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 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). |
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Keywords: | adjuvant chemotherapy amyloid light‐chain amyloidosis bortezomib cirrhosis dexamethasone liver transplant |
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