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遗传性血色素沉着症患者十二指肠末端巨噬细胞缺失及浆细胞性铁储存的存在。

Absence of macrophage and presence of plasmacellular iron storage in the terminal duodenum of patients with hereditary haemochromatosis.

作者信息

Düllmann J, Wulfhekel U, Mohr A, Riecken K, Hausmann K

机构信息

Abteilung für Neuroanatomie, Universitätskrankenhauses Hamburg-Eppendorf, Federal Republic of Germany.

出版信息

Virchows Arch A Pathol Anat Histopathol. 1991;418(3):241-7. doi: 10.1007/BF01606062.

Abstract

Biopsy specimens of the terminal duodenum obtained from 11 patients with hereditary haemochromatosis were examined by light and electron microscopy. Stainable iron was found in the lamina propria of the terminal duodenum in only 4 patients, all of whom were in an advanced stage of the disease. The iron was localized in the basal parts of the villi, sparing their tips, and between the crypts of Lieberkühn. The iron-storing cells could be identified as plasma cells, in which ferritin and haemosiderin were localized within lysosomes and ferritin molecules scattered in the cell sap. There was no storage of iron in macrophages. These observations demonstrate the impaired iron-storing capacity of macrophages in hereditary haemochromatosis, which may be related to the increased iron absorption in this iron storage disease.

摘要

对11例遗传性血色素沉着症患者的十二指肠末端活检标本进行了光镜和电镜检查。仅在4例患者的十二指肠末端固有层中发现了可染色铁,所有这些患者均处于疾病晚期。铁位于绒毛的基部,其尖端未受累,且位于利伯kühn隐窝之间。储铁细胞可鉴定为浆细胞,其中铁蛋白和含铁血黄素位于溶酶体内,铁蛋白分子散布在细胞液中。巨噬细胞中没有铁储存。这些观察结果表明,遗传性血色素沉着症中巨噬细胞的储铁能力受损,这可能与这种铁储存疾病中铁吸收增加有关。

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