-Case Reports-

Hemochromatosis and Hepatocellular Carcinoma Secondary to Immunoglobulin G4-Related Disease with Hepatopathy: A Case Report

Shintaro Kanaka¹, Youichi Kawano², Shigeki Yokomuro², Fumihiko Ando², Norio Itokawa³, Tsutomu Hatori⁴, Koshi Matsumoto⁵, Yoh Zen⁶, Masao Miyashita² and Hiroshi Yoshida¹

¹Department of Surgery, Nippon Medical School, Tokyo, Japan
²Department of Surgery, Nippon Medical School Chiba Hokusoh Hospital, Chiba, Japan
³Division of Gastroenterology, Department of Internal Medicine, Nippon Medical School Chiba Hokusoh Hospital, Chiba, Japan
⁴Department of Clinical Pathology, Nippon Medical School Chiba Hokusoh Hospital, Chiba, Japan
⁵Department of Pathology, Ebina General Hospital, Kanagawa, Japan
⁶Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Hyogo, Japan

Immunoglobulin G4-related disease (IgG4-RD) is a recently characterized illness in which lymphocytes and plasma cells infiltrate various anatomical sites. IgG4-hepatopathy, a 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) developing from IgG4-hepatopathy. A 67-year-old man was admitted to our hospital for treatment of deteriorating glucose tolerance. Blood test results showed hypergammaglobulinemia, especially IgG4. He was readmitted 2 months later with dyspnea due to lung disease and pleural effusion, and elevated transaminase levels. He underwent liver and lung biopsies. IgG4-RD was diagnosed and he was treated with steroid therapy, which improved serum IgG4 levels and imaging abnormalities. A follow-up computed tomography (CT) scan conducted 38 months later revealed a tumor (diameter, 50 mm) in liver segments 7 and 8. The resected specimen revealed HCC and abundant siderosis in the background liver, indicating a diagnosis of hemochromatosis. IgG4-positive cells were scarce, probably because of corticosteroid therapy. In the present case, IgG4-RD was well controlled with prednisolone (PSL) and an immunosuppressive agent, and chronic hepatitis was not severe, even though the patient subsequently developed HCC. However, extensive siderosis consistent with hemochromatosis was unexpectedly noted. These findings suggest that secondary hemochromatosis and HCC developed during IgG4-RD with hepatopathy. We believe this case sheds light on IgG4-RD.

J Nippon Med Sch 2021; 88: 138-144

Keywords
IgG4-related disease, IgG4-hepatopathy, secondary hemochromatosis, hepatocellular carcinoma

Correspondence to
Shintaro Kanaka, Department of Surgery, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
ks32814@nms.ac.jp

Received, March 17, 2020
Accepted, July 2, 2020