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 language | English |
|---|---|
| Pages (from-to) | 386-389 |
| Number of pages | 4 |
| Journal | Clinical Journal of Gastroenterology |
| Volume | 6 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - Oct 1 2013 |
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All Science Journal Classification (ASJC) codes
- Gastroenterology
Cite this
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.
In: Clinical Journal of Gastroenterology, Vol. 6, No. 5, 01.10.2013, p. 386-389.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - A case of fatal intrahepatic cholestasis with primary AL amyloidosis
T2 - Is early diagnosis possible?
AU - Takao, Shinichiro
AU - Tanaka, Kosuke
AU - Miyazaki, Masayuki
AU - Tanaka, Masatake
AU - Ohashi, Tomoko
AU - Kato, Masaki
AU - Kotoh, Kazuhiro
AU - Aishima, Shinichi
AU - Takayanagi, Ryoichi
PY - 2013/10/1
Y1 - 2013/10/1
N2 - 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.
AB - 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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UR - http://www.scopus.com/inward/citedby.url?scp=84888203598&partnerID=8YFLogxK
U2 - 10.1007/s12328-013-0406-x
DO - 10.1007/s12328-013-0406-x
M3 - Article
AN - SCOPUS:84888203598
VL - 6
SP - 386
EP - 389
JO - Clinical Journal of Gastroenterology
JF - Clinical Journal of Gastroenterology
SN - 1865-7257
IS - 5
ER -