Cho Myung Hyun, Park Hee Sun, Han Hye Seung, Bae Sun Hwan
Departments of Pediatrics, Konkuk University School of Medicine, Seoul, Korea.
Department of Radiology, Konkuk University School of Medicine, Seoul, Korea.
Pediatr Int. 2016 Jul;58(7):628-31. doi: 10.1111/ped.12874. Epub 2016 Feb 3.
Giant cell hepatitis (GCH) with autoimmune hemolytic anemia (AHA) is a very rare disease characterized by early onset and severe clinical manifestations, including immune hemolytic anemia and hepatitis with cholestasis. The prognosis is poor despite aggressive immunosuppressive therapy. We report here the first case of GCH with AHA in East Asia. A 2-month-old boy was admitted with jaundice. Blood test indicated abnormal liver function and low hemoglobin. Direct Coombs test and several autoantibodies associated with liver disease were positive, and liver biopsy was consistent with GCH. He was treated with prednisolone and ursodeoxycholic acid, and at the time of writing was in clinical and biochemical remission after prednisolone was stopped.
伴有自身免疫性溶血性贫血（AHA）的巨细胞性肝炎（GCH）是一种非常罕见的疾病，其特征为起病早且临床表现严重，包括免疫性溶血性贫血和伴有胆汁淤积的肝炎。尽管进行了积极的免疫抑制治疗，但其预后仍然很差。我们在此报告东亚地区首例伴有AHA的GCH病例。一名2个月大的男婴因黄疸入院。血液检查显示肝功能异常且血红蛋白水平低。直接抗人球蛋白试验及几种与肝病相关的自身抗体呈阳性，肝脏活检结果符合GCH。他接受了泼尼松龙和熊去氧胆酸治疗，在撰写本文时，停用泼尼松龙后处于临床和生化缓解状态。
