Shinjo K, Takeshita A, Naito K, Ohnishi K, Hirabayashi N, Ohno R
Department of Medicine III, Hamamatsu University School of Medicine.
Rinsho Ketsueki. 2001 Jul;42(7):571-4.
A 33 year-old female patient presented with apparent skin pigmentation, sustained liver dysfunction and impaired glucose tolerance. She had received blood transfusions totalling more than 40,000 ml for myelodysplastic syndrome and an allogeneic bone marrow transplant from her HLA-matched sister at the age of 31. Ferrokinetic data showed a significant iron overload. Magnetic resonance imaging suggested excessive iron deposition in the liver. The patient was diagnosed as having secondary hemochromatosis. She was given subcutaneous injections of 6,000 units of recombinant human erythropoietin initially twice a week and then weekly, and phlebotomies were performed to maintain her hemoglobin level above 10 g/dl. Three years later, the total volume of phlebotomized blood reached 24,000 ml, and her ferrokinetic data, serum transaminase levels, glucose tolerance and skin color were significantly improved.
一名33岁女性患者出现明显的皮肤色素沉着、持续性肝功能不全和糖耐量受损。她因骨髓增生异常综合征接受了总计超过40000毫升的输血,并在31岁时接受了来自其HLA匹配姐姐的异基因骨髓移植。铁动力学数据显示有明显的铁过载。磁共振成像提示肝脏中铁沉积过多。该患者被诊断为继发性血色素沉着症。最初她每周接受两次皮下注射6000单位重组人促红细胞生成素,之后改为每周一次,并进行放血以维持其血红蛋白水平高于10克/分升。三年后,放血总量达到24000毫升,她的铁动力学数据、血清转氨酶水平、糖耐量和肤色都有显著改善。