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所谓的膜性囊肿性病变(MCL)——类蜡样脂色素的一种新变体。

So-called membranocystic lesion (MCL)--a new variant of ceroid type lipopigment.

作者信息

Elleder M

机构信息

1st Department of Pathology, Charles University, School of Medicine, Prague, Czechoslovakia.

出版信息

Adv Exp Med Biol. 1989;266:191-206; discussion 207. doi: 10.1007/978-1-4899-5339-1_14.

Abstract

Structures very close morphologically to the so-called membranocystic formations of the Nasu-Hakola's disease and identical in histochemical properties with them were found in several other metabolically unrelated conditions such as cerebrotendinous xanthomatosis (perivascularly in the brain) and in human atheromatous plaques. This with some other literary data points to unspecific nature of the membranocystic lesion (MCL) which also has been resisting satisfactory classification in terms of pathobiochemistry. Evidence is presented suggesting the MCL is lipopigment in nature. This is based on its lipid histochemical properties dominated by prominent autofluorescence and marked sudanophilia resistant to lipid extraction procedures. Ultrastructural pattern of the MCL was membranous, being dominated by mostly individual trilaminar membranes about 15 nm thick which could be also occasionally identified in various intralysosomal ceroid type lipopigments. It is supposed that the MCL lipopigment is formed mainly extracellularly from the lipid rich debris.

摘要

在其他几种与代谢无关的病症中,如脑腱黄瘤病(在脑内血管周围)和人类动脉粥样硬化斑块中,发现了一些结构在形态上与纳苏 - 哈科拉病的所谓膜性囊肿结构非常相似,并且在组织化学性质上与其相同。这与其他一些文献数据表明膜性囊肿病变(MCL)具有非特异性性质,其在病理生物化学方面也一直难以得到令人满意的分类。有证据表明MCL本质上是脂色素。这是基于其脂质组织化学性质,其主要特征是显著的自发荧光和对脂质提取程序有抗性的明显苏丹ophilia。MCL的超微结构模式是膜性的,主要由大多单个约15nm厚的三层膜组成,这些膜偶尔也可在各种溶酶体内类蜡样脂色素中识别。据推测,MCL脂色素主要由富含脂质的碎片在细胞外形成。

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