Weinstein David A, Roy Cindy N, Fleming Mark D, Loda Massimo F, Wolfsdorf Joseph I, Andrews Nancy C
Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Blood. 2002 Nov 15;100(10):3776-81. doi: 10.1182/blood-2002-04-1260. Epub 2002 Jun 28.
The anemia of chronic disease is a prevalent, poorly understood condition that afflicts patients with a wide variety of diseases, including infections, malignancies, and rheumatologic disorders. It is characterized by a blunted erythropoietin response by erythroid precursors, decreased red blood cell survival, and a defect in iron absorption and macrophage iron retention, which interrupts iron delivery to erythroid precursor cells. We noted that patients with large hepatic adenomas had severe iron refractory anemia similar to that observed in anemia of chronic disease. This anemia resolved spontaneously after adenoma resection or liver transplantation. We investigated the role of the adenomas in the pathogenesis of the anemia and found that they produce inappropriately high levels of hepcidin mRNA. Hepcidin is a peptide hormone that has been implicated in controlling the release of iron from cells. We conclude that hepcidin plays a major, causative role in the anemia observed in our subgroup of patients with hepatic adenomas, and we speculate that it is important in the pathogenesis of the anemia of chronic disease in general.
慢性病贫血是一种普遍存在但了解不足的病症,折磨着患有多种疾病的患者,包括感染、恶性肿瘤和风湿性疾病。其特征是红系前体细胞对促红细胞生成素的反应减弱、红细胞存活时间缩短,以及铁吸收和巨噬细胞铁潴留缺陷,这会中断铁向红系前体细胞的输送。我们注意到,患有大肝腺瘤的患者存在严重的铁难治性贫血,类似于在慢性病贫血中观察到的情况。这种贫血在腺瘤切除或肝移植后会自发缓解。我们研究了腺瘤在贫血发病机制中的作用,发现它们产生的铁调素mRNA水平异常高。铁调素是一种肽激素,与控制细胞中铁的释放有关。我们得出结论,铁调素在我们患有肝腺瘤的患者亚组中观察到的贫血中起主要的致病作用,并且我们推测它在一般慢性病贫血的发病机制中也很重要。