Tabata Sumie, Mori Minako, Nagai Yuya, Hashimoto Hisako, Arima Hiroshi, Nagano Seiji, Takiuchi Yoko, Inoue Daichi, Kimura Takaharu, Shimoji Sonoko, Yanagita Soshi, Ito Kiminari, Matsushita Akiko, Nagai Kenichi, Takahashi Takayuki
Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital.
Intern Med. 2010;49(5):453-6. doi: 10.2169/internalmedicine.49.2991. Epub 2010 Mar 1.
A 21-year-old man who was diagnosed with Diamond-Blackfan anemia at 2 years of age came to our hospital with the hope of undergoing bone marrow transplantation (BMT). He had been red cell transfusion-dependent for about 8 years; at presentation he had transfusion-induced hemochromatosis, a subsequent low left ventricular ejection fraction (LVEF) of 43%, and diabetes mellitus requiring insulin therapy. He received unrelated BMT with reduced intensity conditioning and sufficient GVHD prophylaxis. Regardless of the cardiac dysfunction, he had an uneventful course during pre- and post-grafting periods, and is currently doing well with an improved LVEF (55%), although he is still insulin dependent.
一名2岁时被诊断为先天性纯红细胞再生障碍性贫血的21岁男性患者，怀着接受骨髓移植（BMT）的希望前来我院就诊。他依赖红细胞输血约8年；就诊时，他患有输血性血色素沉着症，随后左心室射血分数（LVEF）降低至43%，并患有需要胰岛素治疗的糖尿病。他接受了低强度预处理和充分移植物抗宿主病（GVHD）预防的非亲缘BMT。尽管存在心脏功能障碍，但他在移植前后过程顺利，目前情况良好，LVEF有所改善（55%），不过仍依赖胰岛素治疗。
