Giant cell hepatitis and autoimmune hemolytic anemia successfully treated with rituximab
Giant cell hepatitis and autoimmune hemolytic anemia successfully treated with rituximab
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DOI：
10.1097/mpg.0b013e31802e9739
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发表时间：
2007-05-01
影响因子：
2.9
通讯作者：
Kerkar, Nanda
中科院分区：
文献类型：
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作者：
Miloh, Tamir;Manwani, Deepa;Kerkar, Nanda
巨细胞性肝炎（GCH）是新生儿胆汁淤积症的常见描述性组织学发现，在新生儿期后罕见。GCH与病毒感染、药物诱导的肝毒性、自身免疫性疾病和其他疾病相关，但并不意味着特定的病因或诊断实体。自身免疫性溶血性贫血（AIHA）的特征是产生针对红细胞表面抗原的抗体，并通过补体和网状内皮系统破坏红细胞，通常是急性和自限性的，对短期类固醇有良好的反应（1）。GCH和AIHA可能作为儿童的独立疾病出现。然而，GCH和AIHA的组合是一种罕见的独特实体，其对免疫抑制治疗的反应较差，并且通常进展为致命的肝脏疾病（1）。我们报告了一名患有GCH和AIHA的婴儿，其肝脏疾病对类固醇，硫唑嘌呤，西罗莫司和静脉注射免疫球蛋白（IVIG）没有反应，但在使用CD20单克隆抗体利妥昔单抗4个疗程后，临床和生化情况有所改善。
Giant cell hepatitis (GCH) is a common descriptive histological finding in infants with neonatal cholestasis and is rare after the neonatal period. GCH has been associated with viral infections, drug-induced hepatotoxicity, autoimmune disorders, and other conditions and does not imply a specific cause or diagnostic entity. Autoimmune hemolytic anemia (AIHA) is characterized by the production of antibodies to red blood cell surface antigens with destruction of red blood cells by complement and reticuloendothelial system and is typically acute and self-limiting, with a good response to a short course of steroids (1). GCH and AIHA may present as independent conditions in children. However, the combination of GCH and AIHA is a rare distinct entity that carries poor response to immunosuppressive therapy and often progresses to fatal liver disease (1). We report on an infant with GCH and AIHA whose liver disease failed to respond to steroids, azathioprine, sirolimus, and intravenous immunoglobulin (IVIG) but improved clinically and biochemically after 4 courses of rituximab, a CD20 monoclonal antibody.