Iancu T C, Neustein H B
Br J Haematol. 1977 Dec;37(4):527-35. doi: 10.1111/j.1365-2141.1977.tb01026.x.
Homozygous beta-thalassaemia is a disease in which there is a progressive iron overload from infancy to death in early adulthood. Liver biopsies from 10 patients in various stages of this disease were examined by electron microscopy. A number of round or oval lysosomal structures, containing lamellae different from myelin figures, were seen in all patients, including those with minimal iron overload. Ferritin molecules were seen either in relationship with the lamellae forming arrays, or in paracrystalline arrangement, or with no organized form. There were practically no ferritin molecules in sub-cellular compartments other than cell sap and lysosomes. The density of cell sap ferritin was constant beyond infancy, but the number of iron-laden lysosomes increased with age. The stages in the process of iron seclusion, seen even in advanced phases of iron overload, are described. Ferritin is thought to accumulate in lysosomes by a transmembraneous movement, but other explanations are considered.
纯合子β地中海贫血是一种从婴儿期到成年早期死亡会逐渐出现铁过载的疾病。对10例处于该疾病不同阶段的患者进行肝脏活检，并通过电子显微镜检查。在所有患者中，包括铁过载程度最低的患者，都观察到了一些圆形或椭圆形的溶酶体结构，其包含与髓鞘样结构不同的板层。铁蛋白分子要么与形成阵列的板层相关，要么呈副晶状排列，要么没有有组织的形式。除了细胞液和溶酶体外，在亚细胞区室中几乎没有铁蛋白分子。婴儿期之后细胞液铁蛋白的密度是恒定的，但含铁溶酶体的数量会随着年龄的增长而增加。本文描述了即使在铁过载的晚期阶段也能看到的铁隔离过程的各个阶段。铁蛋白被认为是通过跨膜运动在溶酶体中积累的，但也考虑了其他解释。
