一例因多次输血导致噬血细胞增多和严重铁过载的恒河猴溶血病病例。
A case of rhesus hemolytic disease with hemophagocytosis and severe iron overload due to multiple transfusions.
作者信息
Yilmaz Sebnem, Duman Nuray, Ozer Esra, Kavas Nazan, Oren Hale, Demircioğlu Fatih, Kumral Abdullah, Ozkan Hasan, Irken Gülersu, Ozer Erdener
机构信息
Department of Pediatric Hematology, Dokuz Eylül University Faculty of Medicine, Izmir, Turkey.
出版信息
J Pediatr Hematol Oncol. 2006 May;28(5):290-2. doi: 10.1097/01.mph.0000212906.07018.93.
BACKGROUND
A newborn with cholestatic hepatic disease and hemophagocytic lymphohistiocytosis due to rhesus hemolytic disease (RHD) is reported.
OBSERVATION
A 34 weeks' gestation baby with RHD, who had received multiple intrauterine transfusions (IUT), developed cholestatic hepatic disease and secondary hemophagocytic lymphohistiocytosis (HLH). Her serum ferritin level increased to 5,527 ng/mL, and liver biopsy showed severe iron overload. Treatment with intravenous desferrioxamine resulted in a marked decrease in serum ferritin levels and normalization of liver function
CONCLUSION
We suggest that patients who have undergone IUT be evaluated for hyperferritinemia. If hyperferritinemia is noted, chelation therapy should be considered. As another rare finding, HLH can complicate the course of RHD.
背景
报道了一例因恒河猴溶血病（RHD）导致胆汁淤积性肝病和噬血细胞性淋巴组织细胞增生症的新生儿。
观察
一名患有RHD的34周妊娠婴儿，接受了多次宫内输血（IUT），出现了胆汁淤积性肝病和继发性噬血细胞性淋巴组织细胞增生症（HLH）。她的血清铁蛋白水平升至5527 ng/mL，肝脏活检显示严重铁过载。静脉注射去铁胺治疗导致血清铁蛋白水平显著下降和肝功能正常化。
结论
我们建议对接受过IUT的患者进行高铁蛋白血症评估。如果发现高铁蛋白血症，应考虑螯合疗法。作为另一个罕见发现，HLH可使RHD病程复杂化。
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