Hemochromatosis and Hepatocellular Carcinoma Secondary to Immunoglobulin G4-Related Disease with Hepatopathy: A Case Report
| Autor: | Shintaro, Kanaka, Youichi, Kawano, Shigeki, Yokomuro, Fumihiko, Ando, Norio, Itokawa, Tsutomu, Hatori, Koshi, Matsumoto, Yoh, Zen, Masao, Miyashita, Hiroshi, Yoshida, Kanaka, Shintaro, Kawano, Youichi, Yokomuro, Shigeki, Ando, Fumihiko, Itokawa, Norio, Hatori, Tsutomu, Matsumoto, Koshi, Zen, Yoh, Miyashita, Masao, Yoshida, Hiroshi |
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| Rok vydání: | 2021 |
| Předmět: |
Male
medicine.medical_specialty Carcinoma Hepatocellular Siderosis Pleural effusion Prednisolone Gastroenterology Internal medicine parasitic diseases medicine Humans skin and connective tissue diseases Hemochromatosis integumentary system business.industry Liver Neoplasms fungi Hypergammaglobulinemia General Medicine Middle Aged medicine.disease Hepatocellular carcinoma Elevated transaminases IgG4-related disease Immunoglobulin G4-Related Disease business Immunosuppressive Agents medicine.drug |
| Zdroj: | Journal of Nippon Medical School. 88:138-144 |
| ISSN: | 1347-3409 1345-4676 |
| DOI: | 10.1272/jnms.jnms.2021_88-306 |
| Popis: | Immunoglobulin G4 (IgG4) -related disease (IgG4-RD) is a recently characterized entity in which lymphocytes and plasma cells infiltrate various anatomical sites. IgG4-hepatopathy, which is the manifestation of IgG4-RD, is a broader term covering various patterns of liver injury. The clinical course including the malignant potential of IgG4-RD remains unclear. Here we report the first case of secondary hemochromatosis and hepatocellular carcinoma (HCC) developed from IgG4-hepatopathy. A 67-year-old man was admitted to our hospital due to deteriorated glucose tolerance. Blood test results showed hypergammaglobulinemia, especially IgG4. He was readmitted 2 months later with dyspnea due to lung disease and pleural effusion, with elevated transaminase levels. He underwent liver and lung biopsies and was diagnosed with IgG4-RD, and received steroid therapy, which improved his serum IgG4 levels and imaging abnormalities. A follow-up computed tomography (CT) scan conducted 38 months later demonstrated a 50-mm-diameter tumor segments 7 and 8 of the liver. The resected specimen revealed HCC and abundant siderosis in the background liver, leading to a diagnosis of hemochromatosis. IgG4-positive cells were scarce, probably due to corticosteroid therapy. In the present case, IgG4-RD was well controlled with prednisolone (PSL) and immunosuppressive agent, and chronic hepatitis was not so severe, even though the patient subsequently developed HCC. However, extensive siderosis consistent with hemochromatosis was unexpectedly noted. It is suggested that secondary hemochromatosis and HCC developed during IgG4-RD with hepatopathy. Here we report the present case because it contributes to our understanding of IgG4-RD. |
| Databáze: | OpenAIRE |
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