Baran Maşallah, Özgenç Funda, Berk Ömer, Gökçe Demir, Kavakli Kaan, Yilmaz Funda, Şen Sait, Yağci Raşit Vural
Department of Pediatric Gastroenterology, Hepatology and Nutrition, Ege University School of Medicine, İzmir.
Turk J Gastroenterol. 2010 Dec;21(4):448-51. doi: 10.4318/tjg.2010.0135.
Autoimmune hemolytic anemia with giant cell hepatitis is a distinct entity in children. It is usually fatal with progressive liver disease. Immunosuppressive treatment with conventional drugs offers some response; however, it is usually only temporary. Alternative therapeutic options with monoclonals have been reported with promising remission of the disease. We report a case with autoimmune hemolytic anemia+giant cell hepatitis after varicella infection. She was resistant to standard immunosuppressive combinations, and rescue therapy with rituximab was used. Remission was not achieved with the drug and the child died with septic complication.
自身免疫性溶血性贫血合并巨细胞肝炎在儿童中是一种独特的疾病实体。它通常会因进行性肝病而致命。使用传统药物进行免疫抑制治疗会有一些反应；然而，通常只是暂时的。据报道，使用单克隆抗体的替代治疗方案有望使疾病缓解。我们报告一例水痘感染后出现自身免疫性溶血性贫血 + 巨细胞肝炎的病例。她对标准免疫抑制联合治疗耐药，因此使用利妥昔单抗进行挽救治疗。该药物未能实现缓解，患儿因败血症并发症死亡。
