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[原发性和继发性血色素沉着症中铁蓄积模式的差异。诊断意义及临床后果]

[Differences in the pattern of iron accumulation in primary and secondary hemochromatosis. Diagnostic importance and clinical consequences].

作者信息

Düllmann J, Wulfhekel U

机构信息

internistischen Gemeinschaftspraxis Lerchenfeld, Hamburg.

出版信息

Folia Haematol Int Mag Klin Morphol Blutforsch. 1990;117(3):413-7.

PMID:1703111
Abstract

In secondary haemochromatosis up to fourfold higher amounts of iron are tolerated in the organism than in primary (hereditary) haemochromatosis. This is connected with the marked iron storage of macrophages in secondary iron overloading, which is relatively without any dangers. In primary haemochromatosis, however, a relative insufficiency of storage of extrahepatic macrophages can be observed for iron, a fact which favours a premature parenchymatous iron storage leading to organ lesions. Because of the discrepant behaviour of macrophages characteristic, diagnostically relevant differences will occur in the pattern of iron storage in the bone-marrow, spleen and small intestine between primary and secondary haemochromatosis.

摘要

在继发性血色素沉着症中,机体耐受的铁量比原发性(遗传性)血色素沉着症高出四倍之多。这与继发性铁过载时巨噬细胞显著的铁储存有关,这种情况相对没有任何危险。然而,在原发性血色素沉着症中,可以观察到肝外巨噬细胞对铁的储存相对不足,这一事实有利于过早的实质细胞铁储存,从而导致器官病变。由于巨噬细胞的行为存在差异,原发性和继发性血色素沉着症在骨髓、脾脏和小肠中的铁储存模式会出现诊断上相关的差异。

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