Activated hepatic stellate cells participate in liver fibrosis in a patient with transfusional iron overload
Activated hepatic stellate cells participate in liver fibrosis in a patient with transfusional iron overload
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DOI：
10.1007/s005350050168
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发表时间：
1998-10-01
影响因子：
6.3
通讯作者：
Kashima, K
中科院分区：
文献类型：
--
作者：
Harada, Y;Iwai, M;Kashima, K
我们描述了一位慢性肾功能衰竭患者长期输血后铁超载引起的肝纤维化。相关实验室数据为：血清-Fe 148 μ g/dl;不饱和铁结合力（UIBC）14 μ g/dl; s-铁蛋白9350 ng/ml;人白细胞抗原（HLA）A2、A24、B39、B55、Cw 1、Cw 7。计算机断层扫描显示肝脏高密度，腹腔镜检查显示棕色肝脏。肝组织学显示来自汇管区的桥接纤维化。在枯否细胞和肝细胞中观察到重铁存款，肝细胞被汇管区周围的纤维化包围。免疫细胞化学显示许多星状细胞中的α-平滑肌肌动蛋白分布沿着纤维化区域，电子显微镜显示浸润的肌纤维母细胞星状细胞与胶原纤维共存于变性肝细胞周围，含铁沉积物。这一发现与星状细胞在遗传性和输血性铁超负荷血色病的肝纤维化中起重要作用的观点一致。
We describe liver fibrosis caused by iron overload after a long history of blood transfusion in a patient with chronic renal failure. Pertinent laboratory data were: serum (s)-Fe 148 mu g/dl; unsaturated iron binding capacity (UIBC) 14 mu g/dl; s-ferritin 9350 ng/ml; human leukocyte antigen (HLA) A2, A24, B39, B55, Cw1, Cw7. Computed tomography revealed a high density in the liver, and laparoscopy revealed a brown liver. Liver histology showed bridging fibrosis from portal tracts. A heavy iron deposit was seen in Kupffer cells as well as in hepatocytes surrounded by fibrosis around the portal tracts. Immunocytochemistry revealed ct-smooth muscle actin in many stellate cells distributed along the fibrotic area, and electron microscopy revealed infiltrating myofibroblastic stellate cells coexisting with collagen fibers around degenerated hepatocytes containing iron deposits. The findings are consistent with the notion that stellate cells play an important role in liver fibrogenesis in both genetic and transfusional iron overload hemochromatosis.