naa a22 4500 510814182 CHVBK 20180411083457.0 cr unu---uuuuu 180411e20131001xx s 000 0 eng 10.1007/s12328-013-0406-x doi (NATIONALLICENCE)springer-10.1007/s12328-013-0406-x A case of fatal intrahepatic cholestasis with primary AL amyloidosis: is early diagnosis possible? [Elektronische Daten] [Shinichiro Takao, Kosuke Tanaka, Masayuki Miyazaki, Masatake Tanaka, Tomoko Ohashi, Masaki Kato, Kazuhiro Kotoh, Shinichi Aishima, Ryoichi Takayanagi] 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 1year 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 1.5months 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. Springer Japan, 2013 Amyloidosis nationallicence Liver failure nationallicence γ-Glutamyltransferase nationallicence Alkaline phosphatase nationallicence Light chain nationallicence Takao Shinichiro Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut Tanaka Kosuke Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut Miyazaki Masayuki Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut Tanaka Masatake Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut Ohashi Tomoko Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut Kato Masaki Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut Kotoh Kazuhiro Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut Aishima Shinichi Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan aut Takayanagi Ryoichi Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut Clinical Journal of Gastroenterology Springer Japan 6/5(2013-10-01), 386-389 1865-7257 6:5<386 2013 6 12328 https://doi.org/10.1007/s12328-013-0406-x text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s12328-013-0406-x text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Takao Shinichiro Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut NATIONALLICENCE 700 1- Tanaka Kosuke Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut NATIONALLICENCE 700 1- Miyazaki Masayuki Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut NATIONALLICENCE 700 1- Tanaka Masatake Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut NATIONALLICENCE 700 1- Ohashi Tomoko Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut NATIONALLICENCE 700 1- Kato Masaki Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut NATIONALLICENCE 700 1- Kotoh Kazuhiro Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut NATIONALLICENCE 700 1- Aishima Shinichi Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan aut NATIONALLICENCE 700 1- Takayanagi Ryoichi Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan aut NATIONALLICENCE 773 0- Clinical Journal of Gastroenterology Springer Japan 6/5(2013-10-01), 386-389 1865-7257 6:5<386 2013 6 12328 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer