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特发性血色素沉着症、地中海贫血和肝脏疾病中的单核细胞铁蛋白

Monocyte ferritin in idiopathic haemochromatosis, thalassaemia and liver disease.

作者信息

Saab G, Green R, Jurjus A, Sarrou E

出版信息

Scand J Haematol. 1986 Jan;36(1):65-70. doi: 10.1111/j.1600-0609.1986.tb02652.x.

Abstract

Monocyte ferritin (MF) content was measured in normal subjects and patients with a variety of disorders of iron storage. MF was above the normal range in 4 patients with idiopathic haemochromatosis (IHC). However, in 4 patients with transfusion siderosis (TS), secondary to aplastic anaemia, who had similar elevations in serum ferritin, MF was highly elevated. 10 patients with thalassaemia intermedia and thalassaemia major with no previous history of transfusions, but with elevated serum ferritin, also had significantly elevated MF. Disproportionately low MF in IHC could reflect defective ferritin metabolism in reticuloendothelial cells in this disorder. Finally, in 3 patients with acute rises in serum ferritin caused by acute hepatitis, MF was not increased. This suggests that MF is not directly affected by high circulating levels of serum ferritin raised acutely, but rather reflects iron storage status in conditions not associated with primary disorders of iron metabolism.

摘要

在正常受试者以及患有各种铁储存障碍疾病的患者中测量了单核细胞铁蛋白(MF)含量。4例特发性血色素沉着症(IHC)患者的MF高于正常范围。然而,在4例继发于再生障碍性贫血的输血性铁沉着症(TS)患者中,其血清铁蛋白有类似升高,但MF却显著升高。10例中间型和重型地中海贫血患者,既往无输血史,但血清铁蛋白升高,其MF也显著升高。IHC中MF不成比例地降低可能反映了该疾病中网状内皮细胞中铁蛋白代谢存在缺陷。最后,在3例因急性肝炎导致血清铁蛋白急性升高的患者中,MF并未升高。这表明MF不受急性升高的高循环血清铁蛋白水平直接影响,而是反映了与铁代谢原发性疾病无关的情况下的铁储存状态。

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