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血色素沉着症、肝癌和红细胞增多症三联征。

The triad of hemochromatosis, hepatoma and erythrocytosis.

作者信息

Raphael B, Cooperberg A A, Niloff P

出版信息

Cancer. 1979 Feb;43(2):690-4. doi: 10.1002/1097-0142(197902)43:2<690::aid-cncr2820430241>3.0.co;2-r.

Abstract

The triad of hemochromatosis, hepatoma and erythrocytosis is a rare combination. Hemochromatosis is often not recognized until the patient presents with the symptoms of hepatocellular carcinoma and erythrocytosis, and the development of erythrocytosis is an important clue to the under-lying hepatoma. The high serum iron concentration and the high saturation of the iron-binding protein, as well as the typical bone marrow hemosiderin pattern, are important aids in the recognition of hemochromatosis. To date, all patients with this triad have been elderly males. The clinical course is usually one of rapid deterioration and death. The seven previously reported cases have been reviewed and the relationship of the erythrocytosis to the increased production of erythropoietin is discussed.

摘要

血色素沉着症、肝癌和红细胞增多症三联征是一种罕见的组合。血色素沉着症往往在患者出现肝细胞癌和红细胞增多症症状时才被发现,而红细胞增多症的出现是潜在肝癌的重要线索。高血清铁浓度、铁结合蛋白的高饱和度以及典型的骨髓含铁血黄素模式,有助于血色素沉着症的识别。迄今为止,所有患有这种三联征的患者均为老年男性。临床病程通常是迅速恶化并死亡。对之前报道的7例病例进行了回顾,并讨论了红细胞增多症与促红细胞生成素产量增加之间的关系。

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