Medical Therapy Unit (Thalassaemia Service), Southern Health, Clayton, Australia.
Blood. 2013 Jul 4;122(1):124-33. doi: 10.1182/blood-2012-12-471441. Epub 2013 May 8.
β-Thalassemia major causes ineffective erythropoiesis and chronic anemia and is associated with iron overload due to both transfused iron and increased iron absorption, the latter mediated by suppression of the iron-regulatory hormone hepcidin. We sought to determine whether, in β-thalassemia major, transfusion-mediated inhibition of erythropoiesis dynamically affects hepcidin. We recruited 31 chronically transfused patients with β-thalassemia major and collected samples immediately before and 4 to 8 days after transfusion. Pretransfusion hepcidin was positively correlated with hemoglobin and ferritin and inversely with erythropoiesis. The hepcidin-ferritin ratio indicated hepcidin was relatively suppressed given the degree of iron loading. Posttransfusion, hemoglobin and hepcidin increased, and erythropoietin and growth differentiation factor-15 decreased. By multiple regression, pre- and posttransfusion hepcidin concentrations were both associated positively with hemoglobin, inversely with erythropoiesis, and positively with ferritin. Although men and women had similar pretransfusion hemoglobin, men had significantly increased erythropoiesis and lower hepcidin, received a lower transfusion volume per liter blood volume, and experienced a smaller posttransfusion reduction in erythropoiesis and hepcidin rise. Age of blood was not associated with posttransfusion hemoglobin or ferritin change. Hepcidin levels in patients with β-thalassemia major dynamically reflect competing influences from erythropoiesis, anemia, and iron overload. Measurement of these indices could assist clinical monitoring.
β-地中海贫血导致无效的红细胞生成和慢性贫血，并伴有铁过载，这是由于输注的铁和增加的铁吸收所致，后者由铁调节激素铁调素的抑制介导。我们试图确定在β-地中海贫血中，输血介导的红细胞生成抑制是否会动态影响铁调素。我们招募了 31 名慢性输血的β-地中海贫血患者，并在输血前和输血后 4 至 8 天采集样本。输血前铁调素与血红蛋白和铁蛋白呈正相关，与红细胞生成呈负相关。铁调素-铁蛋白比值表明，鉴于铁负荷程度，铁调素相对受到抑制。输血后，血红蛋白和铁调素增加，促红细胞生成素和生长分化因子 15 减少。通过多元回归，输血前和输血后铁调素浓度均与血红蛋白呈正相关，与红细胞生成呈负相关，与铁蛋白呈正相关。尽管男性和女性的输血前血红蛋白相似，但男性的红细胞生成明显增加，铁调素降低，每单位血容量输注的输血体积较低，输血后红细胞生成和铁调素升高减少。血龄与输血后血红蛋白或铁蛋白变化无关。β-地中海贫血患者的铁调素水平动态反映了红细胞生成、贫血和铁过载的竞争影响。这些指标的测量可以协助临床监测。
