IRCCS Ca' Granda Foundation Maggiore Policlinico Hospital, University of Milan, Milan, Italy.
Blood Rev. 2012 Apr;26 Suppl 1:S16-9. doi: 10.1016/S0268-960X(12)70006-1.
Iron overload due to increased intestinal iron absorption represents an important clinical problem in patients with non-transfusion-dependent thalassemia (NTDT), particularly as they advance in age. Current models for iron metabolism in patients with beta (β)-thalassemia intermedia (TI) suggest that suppression of serum hepcidin results in increased iron absorption and release of iron from the reticuloendothelial system, leading to depletion of macrophage iron, relatively low levels of serum ferritin, and liver iron loading. The clinical consequences of iron overload in patients with NTDT are multifactorial and include endocrinopathy, bone disease, thromboembolism, pulmonary hypertension, cerebrovascular and neuronal damage, liver fibrosis or cirrhosis, and increased risk of hepatocellular carcinoma. Although serum ferritin levels correlate with liver iron concentration (LIC), they underestimate iron load in these patients compared with transfusion-dependent patients with equivalent LIC. Therefore, direct measurement of LIC is recommended with chelation therapy as indicated.
由于肠道铁吸收增加导致的铁过载是无输血依赖型地中海贫血（NTDT）患者的一个重要临床问题，尤其是随着他们年龄的增长。目前对β地中海贫血中间型（TI）患者铁代谢的模型表明，血清hepcidin 的抑制导致铁吸收增加和网状内皮系统中铁的释放，导致巨噬细胞铁耗竭、血清铁蛋白水平相对较低以及肝脏铁负荷增加。NTDT 患者铁过载的临床后果是多因素的，包括内分泌病、骨骼疾病、血栓栓塞、肺动脉高压、脑血管和神经元损伤、肝纤维化或肝硬化以及肝细胞癌风险增加。虽然血清铁蛋白水平与肝脏铁浓度（LIC）相关，但与具有相同 LIC 的依赖输血的患者相比，它们低估了这些患者的铁负荷。因此，建议在有指征时通过螯合疗法直接测量 LIC。
