Division of Cardiology, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
J Microsc. 2010 Jun 1;238(3):265-74. doi: 10.1111/j.1365-2818.2009.03355.x.
Hepatic iron overload is a common clinical problem resulting from hyperabsorption syndromes and from chronic transfusion therapy. Not only does iron loading vary between reticuloendothelial stores and hepatocytes, but iron is heterogeneously distributed within hepatocytes as well. Since the accessibility of iron particles to chelation may depend, in part, on their distribution, we sought to characterize the shape and scale of iron deposition in humans with transfusional iron overload. Toward this end, we performed a histological analysis of iron stores in liver biopsy specimens of 20 patients (1.3-57.8 mg iron/g dry tissue weight) with aid of electron and light microscopy. We estimated distributions related to variability in siderosomal size, proximity of iron centres and inter-cellular iron loading. These distributions could be well modelled by Gamma distribution functions over most of the pathologic range of iron concentrations. Thus, for a given liver iron burden, a virtual iron-overloaded liver could be created that served as a model for the true histologic appearance. Such a model may be helpful for understanding the mechanics of iron loading or in predicting response to iron removal therapy.
肝脏铁过载是一种常见的临床问题，源于铁吸收过多综合征和慢性输血治疗。不仅网状内皮细胞储存铁和肝细胞中铁的负载情况不同，而且肝细胞内铁的分布也不均匀。由于铁颗粒对螯合的可及性可能部分取决于其分布，我们试图描述输血性铁过载患者肝脏中铁沉积的形状和大小。为此，我们通过电子和光显微镜对 20 名患者（肝组织干重中含铁 1.3-57.8mg）的肝活检标本中铁储存进行了组织学分析。我们通过对类铁体大小、铁中心的接近程度和细胞间铁负荷的变异性进行了估计。这些分布在大部分病理铁浓度范围内都可以用伽马分布函数很好地建模。因此，对于给定的肝铁负荷，可以创建一个虚拟的铁过载肝脏模型，作为真实组织学表现的模型。这种模型可能有助于理解铁负荷的机制，或预测对铁去除治疗的反应。
