Demircioğlu Fatih, Çağlayan Sözmen Şule, Yılmaz Şebnem, Ören Hale, Arslan Nur, Kumral Abdullah, Özer Erdener, İrken Gülersu
Dörtçelik Children's Hospital, Bursa, Turkey, Phone: +90 505 525 03 47 E-mail:
Turk J Haematol. 2010 Sep 5;27(3):204-8. doi: 10.5152/tjh.2010.30.
A 33 weeks' gestation, a baby with rhesus hemolytic disease (RHD), who had received intrauterine transfusions twice, developed cholestatic hepatic disease and late hyporegenerative anemia. Her serum ferritin and bilirubin levels increased to 8842 ng/ml and 17.9 mg/dl, respectively. Liver biopsy showed cholestasis and severe iron overload. Treatment with recombinant erythropoietin (rHuEPO) decreased the transfusion need, and intravenous deferoxamine resulted in a marked decreased in serum ferritin levels and normalization of liver function. In patients who have undergone intrauterine transfusions due to RHD, hyperferritinemia and late hyporegenerative anemia should be kept in mind. Chelation therapy in cases with symptomatic hyperferritinemia and rHuEPO treatment in cases with severe hyporegenerative anemia should be considered.
一名妊娠33周的患有恒河猴溶血病(RHD)的婴儿接受了两次宫内输血,出现了胆汁淤积性肝病和晚期再生低下性贫血。她的血清铁蛋白和胆红素水平分别升至8842 ng/ml和17.9 mg/dl。肝脏活检显示胆汁淤积和严重的铁过载。重组促红细胞生成素(rHuEPO)治疗减少了输血需求,静脉注射去铁胺导致血清铁蛋白水平显著下降且肝功能恢复正常。对于因RHD接受过宫内输血的患者,应牢记高铁蛋白血症和晚期再生低下性贫血。对于有症状的高铁蛋白血症病例应考虑螯合疗法,对于严重再生低下性贫血病例应考虑rHuEPO治疗。