人体组织与器官内有大量铁质沉着而无任何明显原因者,称为原发性血色病(IHC),据此可与外因性铁负荷过度(如反复的输血)所致的继发性血色病相区别。原发性血色病病程延续多年,后期发展为肝硬变。本病实验室检查以血清铁浓度明显升高以及血清转铁蛋白(transferrin)饱和度增高为特征,且早期即 In humans, there is a substantial amount of iron found in the tissues and organs without any apparent cause, known as idiopathic hemochromatosis (IHC), which is associated with secondary hemoglobin due to extrinsic iron overload (eg, repeated transfusions) Difference between the disease. The duration of primary hemochromatosis lasted for many years, the late development of cirrhosis. Laboratory tests of this disease are characterized by a marked increase in serum iron concentration and an increase in serum saturation of transferrin, and that early