Deugnier Y, Turlin B, le Quilleuc D, Moirand R, Loréal O, Messner M, Meunier B, Brissot P, Launois B
Clinique des Maladies du Foie, Hôpital Pontchaillou, Rennes, France.
Am J Surg Pathol. 1997 Jun;21(6):669-75. doi: 10.1097/00000478-199706000-00007.
The aim of this study was to describe the histologic pattern of iron distribution in end-stage cirrhosis due to various causes and to test the reliability of the hepatic iron index (equal to hepatic iron concentration divided by age) in excluding or confirming associated hemochromatosis in such a condition. Large slices of the resected livers of 30 patients transplanted for alcoholic and/or viral end-stage cirrhosis were assessed histologically for iron distribution and biochemically for hepatic iron concentration in the least and the most iron-overloaded nodules of each case. HLA-A3 was used as the marker for the hemochromatosis gene in the population studied. Intranodular parenchymal siderosis was found in 23 cases (12 spotty, 11 diffuse) with diffuse intrabiliary iron deposits apparent in only two cases. Although in 14 patients the hepatic iron index was significantly high (> 1.9) so as to suggest hemochromatosis, these cases did not correspond to homozygous hemochromatosis with respect to the prevalence of HLA-A3 antigen. End-stage cirrhosis arising from different causes is frequently complicated by parenchymal siderosis that may mimic hemochromatosis, including a hepatic iron index greater than 1.9. The diagnosis of hemochromatosis in patients with end-stage cirrhosis, even those with a hepatic iron index greater than 1.9, should rely mainly on clinical and histologic data.
本研究的目的是描述各种病因所致终末期肝硬化中铁分布的组织学模式，并检验肝铁指数（等于肝铁浓度除以年龄）在排除或确诊此类疾病中相关血色素沉着症方面的可靠性。对30例因酒精性和/或病毒性终末期肝硬化而接受肝移植患者的切除肝脏大切片进行组织学评估以确定铁分布，并对每例中含铁最少和最多的结节进行肝铁浓度生化检测。在研究人群中，HLA - A3用作血色素沉着症基因的标志物。23例（12例为散在性，11例为弥漫性）发现有结节内实质细胞铁质沉着，仅2例有明显的弥漫性胆管内铁沉积。虽然14例患者的肝铁指数显著升高（>1.9），提示血色素沉着症，但就HLA - A3抗原的流行情况而言，这些病例并不符合纯合子血色素沉着症。不同病因引起的终末期肝硬化常并发实质细胞铁质沉着，可能酷似血色素沉着症，包括肝铁指数大于1.9。终末期肝硬化患者，即使肝铁指数大于1.9，血色素沉着症的诊断也应主要依靠临床和组织学资料。
