Takahashi Hiroyoshi, Koh Katsuyoshi, Yasui Naoko, Mori Makiko, Akiyama Kosuke, Seki Masafumi, Kato Motohiro, Kagimoto Seiichi, Oh-Ishi Tsutomu, Hanada Ryoji
Department of Hematology/Oncology, Saitama Children's Medical Center, Japan.
Rinsho Ketsueki. 2012 Nov;53(11):1926-31.
We report a case of a 15-year-old girl with severe aplastic anemia who underwent orthotopic liver transplantation 5 years ago for fulminant hepatic failure during the course of immunodeficiency of unknown etiology. She previously exhibited similar immunodeficiency and experienced recurrent viral infections. She developed jaundice at 9 years of age and was diagnosed with fulminant hepatitis. One month later, she underwent living donor liver transplantation, with the donor being her father. Five years after the liver transplant, pancytopenia was noted; she did not respond to treatment with increasing doses of tacrolimus/prednisone and administration of granulocyte-colony stimulating factor. Bone marrow biopsy was performed, and severe aplastic anemia was diagnosed. Six years after the liver transplant, she underwent bone marrow transplantation (BMT), with the donor being her HLA-matched sibling. However, she developed liver dysfunction with recovery of white blood cells. She developed sepsis, which eventually led to her death on day 30 after BMT.
我们报告一例15岁患有严重再生障碍性贫血的女孩，她在5年前因病因不明的免疫缺陷过程中出现暴发性肝衰竭而接受了原位肝移植。她之前表现出类似的免疫缺陷并经历了反复的病毒感染。她9岁时出现黄疸，被诊断为暴发性肝炎。1个月后，她接受了活体供肝肝移植，供体是她的父亲。肝移植5年后，发现全血细胞减少；她对增加剂量的他克莫司/泼尼松治疗以及粒细胞集落刺激因子的给药无反应。进行了骨髓活检，诊断为严重再生障碍性贫血。肝移植6年后，她接受了骨髓移植（BMT），供体是与她HLA匹配的同胞。然而，她出现了肝功能障碍，同时白细胞恢复。她发生了败血症，最终在BMT后第30天死亡。
