Idiopathic copper toxicosis: is abnormal copper metabolism a primary cause of this disease?

• Published in: Medical molecular morphology 2020-03, Vol.53 (1), p.50-55
• Main Authors: Harada, Masaru, Honma, Yuichi, Yoshizumi, Tomoharu, Kumamoto, Keiichiro, Oe, Shinji, Harada, Noboru, Tanimoto, Aya, Yabuki, Kei, Karasuyama, Tsukasa, Yoneda, Akitoshi, Shibata, Michihiko
• Format: Article
• Language: English
• Subjects: Anatomy; Biopsy; Carcinoma, Hepatocellular - drug therapy; Carcinoma, Hepatocellular - metabolism; Carcinoma, Hepatocellular - pathology; Carcinoma, Hepatocellular - surgery; Case Report; Ceruloplasmin - metabolism; Chelating Agents - therapeutic use; Chemical and Drug Induced Liver Injury - drug therapy; Chemical and Drug Induced Liver Injury - metabolism; Chemical and Drug Induced Liver Injury - pathology; Chemical and Drug Induced Liver Injury - surgery; Copper; Copper - metabolism; Copper - toxicity; Female; Hepatocytes - metabolism; Hepatocytes - pathology; Hepatolenticular Degeneration - drug therapy; Hepatolenticular Degeneration - metabolism; Hepatolenticular Degeneration - pathology; Hepatolenticular Degeneration - surgery; Humans; Liver; Liver - metabolism; Liver - pathology; Liver - surgery; Liver cancer; Liver Cirrhosis - drug therapy; Liver Cirrhosis - metabolism; Liver Cirrhosis - pathology; Liver Cirrhosis - surgery; Liver Neoplasms - drug therapy; Liver Neoplasms - metabolism; Liver Neoplasms - pathology; Liver Neoplasms - surgery; Liver Transplantation; Medicine; Medicine & Public Health; Metabolism; Metal Metabolism, Inborn Errors - drug therapy; Metal Metabolism, Inborn Errors - metabolism; Metal Metabolism, Inborn Errors - pathology; Metal Metabolism, Inborn Errors - surgery; Molecular Medicine; Pathology; Toxicosis; Trientine - therapeutic use; Wilson's disease; Young Adult
• ISSN: 1860-1480; 1860-1499
• DOI: 10.1007/s00795-019-00227-4

Idiopathic copper toxicosis (ICT) is characterized by marked copper deposition, Mallory-Denk body (MDB) formation and severe hepatic injury. Although the characteristics are apparently different from Wilson disease, large amounts of copper accumulate in the liver of the patients. We extensively treated a patient with ICT to reduce the body copper, however, the patient needed liver transplantation. Previous liver biopsy revealed high copper content. But extirpated liver contained an extremely small amount of copper, although MDBs and severe inflammation remained. These phenomena suggest abnormal copper metabolism is not the principle cause of ICT but some other abnormality must exist.