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

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)386-389
Number of pages4
JournalClinical Journal of Gastroenterology
Volume6
Issue number5
DOIs
Publication statusPublished - Oct 2013

All Science Journal Classification (ASJC) codes

  • Gastroenterology

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