Pathology of hepatic iron overload
Pathology of hepatic iron overload
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DOI：
10.3748/wjg.v13.i35.4755
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发表时间：
2007-09-21
影响因子：
4.3
通讯作者：
Turlin, Bruno
中科院分区：
文献类型：
--
作者：
Deugnier, Yves;Turlin, Bruno
虽然影像学和遗传学的进步使得大多数遗传性铁超载病例的非侵入性诊断成为可能，但肝脏病理学仍然经常有用（1）通过分级纤维化和寻找相关病变来评估预后，（2）指导病因诊断，特别是当没有分子标记物时。那么，肝铁质沉着症的类型（实质、间充质或混合）及其在整个小叶和肝脏中的分布是提示其病因的有用手段：HLA连锁血色素沉着症基因（HFE）血色素沉着症或其他罕见的遗传性血色素沉着症，非血色素沉着性遗传性铁过载（膜铁转运蛋白病，浆细胞蛋白血症）或继发于过量铁供应的铁过载，炎性综合征，非酒精性慢性肝病，包括代谢异常铁过载综合征、肝硬化和血液病。(C)2007年WJG。All rights reserved.
Although progress in imaging and genetics allow for a noninvasive diagnosis of most cases of genetic iron overload, liver pathology remains often useful (1) to assess prognosis by grading fibrosis and seeking for associated lesions and (2) to guide the etiological diagnosis, especially when no molecular marker is available. Then, the type of liver siderosis (parenchymal, mesenchymal or mixed) and its distribution throughout the lobule and the liver are useful means for suggesting its etiology: HLA-linked hemochromatosis gene (HFE) hemochromatosis or other rare genetic hemochromatosis, nonhemochromatotic genetic iron overload (ferroportin disease, aceruloplasminemia), or iron overload secondary to excessive iron supply, inflammatory syndrome, noncirrhotic chronic liver diseases including dysmetabolic iron overload syndrome, cirrhosis, and blood disorders. (C) 2007 WJG. All rights reserved.