A case of fatal intrahepatic cholestasis with primary AL amyloidosis: Is early diagnosis possible?

Shinichiro Takao, Kosuke Tanaka, Masayuki Miyazaki, Masatake Tanaka, Tomoko Ohashi, Masaki Kato, Kazuhiro Kotoh, Shinichi Aishima, Ryoichi Takayanagi

研究成果: ジャーナルへの寄稿記事

抄録

Immunoglobulin light chain-associated (AL) amyloidosis is a multisystemic disorder characterized by extracellular deposition of immunoglobulin light chain produced by a proliferative plasma cell clone. Although the liver is the major organ involved in AL amyloidosis, hepatic involvement is often clinically asymptomatic and severe intrahepatic cholestasis as the primary manifestation of the disease is rare. A 60-year-old man with severe jaundice, massive ascites and highly elevated alkaline phosphatase was diagnosed with AL amyloidosis by a transjugular liver biopsy. He had undergone a yearly medical check that showed no abnormalities except for mild elevation of serum γ-glutamyltransferase at 1 year before admission. Owing to his poor condition and rapidly progressive liver and renal dysfunction, neither stem cell transplantation nor a combination of chemotherapeutic agents could be applied, and he died 1.5 months after admission. An autopsy revealed amyloid deposition in the systemic organs, and there was no evidence of multiple myeloma. Continuous elevation of γ-glutamyltransferase may be a useful marker for early diagnosis of fatal hepatic amyloidosis.

元の言語英語
ページ(範囲)386-389
ページ数4
ジャーナルClinical Journal of Gastroenterology
6
発行部数5
DOI
出版物ステータス出版済み - 10 1 2013

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Amyloidosis
Early Diagnosis
Immunoglobulin Light Chains
Liver
Intrahepatic Cholestasis
Stem Cell Transplantation
Rare Diseases
Jaundice
Plasma Cells
Multiple Myeloma
Ascites
Amyloid
Alkaline Phosphatase
Liver Diseases
Autopsy
Clone Cells
Kidney
Biopsy
Progressive familial intrahepatic 1 Cholestasis
Primary amyloidosis

All Science Journal Classification (ASJC) codes

  • Gastroenterology

これを引用

A case of fatal intrahepatic cholestasis with primary AL amyloidosis : Is early diagnosis possible? / Takao, Shinichiro; Tanaka, Kosuke; Miyazaki, Masayuki; Tanaka, Masatake; Ohashi, Tomoko; Kato, Masaki; Kotoh, Kazuhiro; Aishima, Shinichi; Takayanagi, Ryoichi.

:: Clinical Journal of Gastroenterology, 巻 6, 番号 5, 01.10.2013, p. 386-389.

研究成果: ジャーナルへの寄稿記事

Takao, Shinichiro ; Tanaka, Kosuke ; Miyazaki, Masayuki ; Tanaka, Masatake ; Ohashi, Tomoko ; Kato, Masaki ; Kotoh, Kazuhiro ; Aishima, Shinichi ; Takayanagi, Ryoichi. / A case of fatal intrahepatic cholestasis with primary AL amyloidosis : Is early diagnosis possible?. :: Clinical Journal of Gastroenterology. 2013 ; 巻 6, 番号 5. pp. 386-389.
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abstract = "Immunoglobulin light chain-associated (AL) amyloidosis is a multisystemic disorder characterized by extracellular deposition of immunoglobulin light chain produced by a proliferative plasma cell clone. Although the liver is the major organ involved in AL amyloidosis, hepatic involvement is often clinically asymptomatic and severe intrahepatic cholestasis as the primary manifestation of the disease is rare. A 60-year-old man with severe jaundice, massive ascites and highly elevated alkaline phosphatase was diagnosed with AL amyloidosis by a transjugular liver biopsy. He had undergone a yearly medical check that showed no abnormalities except for mild elevation of serum γ-glutamyltransferase at 1 year before admission. Owing to his poor condition and rapidly progressive liver and renal dysfunction, neither stem cell transplantation nor a combination of chemotherapeutic agents could be applied, and he died 1.5 months after admission. An autopsy revealed amyloid deposition in the systemic organs, and there was no evidence of multiple myeloma. Continuous elevation of γ-glutamyltransferase may be a useful marker for early diagnosis of fatal hepatic amyloidosis.",
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AU - Aishima, Shinichi

AU - Takayanagi, Ryoichi

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