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具有抗转铁蛋白活性的单克隆免疫球蛋白:转铁蛋白饱和度增加导致高铁血症的罕见原因。

Monoclonal immunoglobulin with antitransferrin activity: A rare cause of hypersideremia with increased transferrin saturation.

作者信息

Alyanakian Marie-Alexandra, Taes Youri, Bensaïd Mimouna, Segovia Berta, Aucouturier Pierre, Rain Jean-Didier, Delanghe Joris, Varet Bruno, Beaumont Carole

机构信息

Université Paris 5 René Descartes; Assistance Publique-Hôpitaux de Paris (AP-HP), Laboratoire d'Immunologie, Hôpital Necker, Paris, France.

出版信息

Blood. 2007 Jan 1;109(1):359-61. doi: 10.1182/blood-2006-05-023762. Epub 2006 Sep 12.

Abstract

Two unusual but similar cases of very high serum iron, extremely high transferrin concentrations (5.4 to 6.5 g/L), and increased transferrin saturation are reported. No anemia or microcytosis was observed. The ferritin concentration remained within the normal range, and no iron overload was observed. In one case, the in vivo half-life of 59Fe-labeled transferrin was shown to be prolonged (206 minutes versus 75 minutes for controls). In both patients, a monoclonal IgG was observed in the serum. The association between serum transferrin and monoclonal IgG was demonstrated by Western blot analysis and affinity chromatography. We suggest that the transferrin bound to the monoclonal IgG molecule has a prolonged half-life in the circulation, leading to high transferrin concentrations, and that the increased serum iron values prevent the onset of anemia. The antitransferrin activity of monoclonal antibody should be added to the list of situations accounting for elevated serum iron with elevated transferrin saturation.

摘要

报告了两例不寻常但相似的病例,血清铁极高,转铁蛋白浓度极高(5.4至6.5g/L),转铁蛋白饱和度增加。未观察到贫血或小红细胞症。铁蛋白浓度保持在正常范围内,未观察到铁过载。在一例病例中,59Fe标记的转铁蛋白的体内半衰期显示延长(206分钟,而对照组为75分钟)。两名患者血清中均观察到单克隆IgG。通过蛋白质印迹分析和亲和层析证明了血清转铁蛋白与单克隆IgG之间的关联。我们认为,与单克隆IgG分子结合的转铁蛋白在循环中的半衰期延长,导致转铁蛋白浓度升高,并且血清铁值升高可防止贫血的发生。单克隆抗体的抗转铁蛋白活性应被列入导致血清铁升高伴转铁蛋白饱和度升高的情况清单中。

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