Harada Y, Iwai M, Kakusui M, Mori T, Tada K, Ishii Y, Okanoue T, Kashima K
Third Department of Internal Medicine, Kyoto Prefectural University of Medicine, Japan.
J Gastroenterol. 1998 Oct;33(5):751-4. doi: 10.1007/s005350050168.
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 microg/dl; unsaturated iron binding capacity (UIBC) 14 microg/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 alpha-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.
我们描述了一名慢性肾衰竭患者在长期输血后因铁过载导致的肝纤维化。相关实验室数据如下：血清铁（s-Fe）148微克/分升；不饱和铁结合力（UIBC）14微克/分升；s-铁蛋白9350纳克/毫升；人类白细胞抗原（HLA）A2、A24、B39、B55、Cw1、Cw7。计算机断层扫描显示肝脏密度增高，腹腔镜检查显示肝脏呈褐色。肝脏组织学检查显示门管区出现桥接纤维化。在库普弗细胞以及门管区周围纤维化所包围的肝细胞中可见大量铁沉积。免疫细胞化学显示，许多星状细胞中存在α平滑肌肌动蛋白，这些星状细胞沿纤维化区域分布，电子显微镜检查显示，在含有铁沉积的变性肝细胞周围，浸润性肌成纤维细胞星状细胞与胶原纤维共存。这些发现与以下观点一致，即星状细胞在遗传性和输血性铁过载血色素沉着症的肝纤维化形成过程中起重要作用。
