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.

中文翻译：

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非输血依赖型地中海贫血中铁超负荷的临床观点。

非肠道依赖型地中海贫血（NTDT）患者尤其是随着年龄的增长，由于肠道铁吸收增加而引起的铁超载是一个重要的临床问题。β-中地中海贫血（TI）患者的铁代谢新模型表明，抑制血清铁调素可增加铁的吸收并从网状内皮系统释放铁，导致巨噬细胞铁耗竭，血清水平相对较低铁蛋白和肝铁负荷。NTDT患者铁超负荷的临床后果是多方面的，包括内分泌病，骨病，血栓栓塞，肺动脉高压，脑血管和神经元损害，肝纤维化或肝硬化以及肝细胞癌风险增加。尽管血清铁蛋白水平与肝铁浓度（LIC）相关，但与具有相同LIC的输血依赖性患者相比，它们低估了这些患者的铁负荷。因此，如所示，建议在螯合疗法下直接测量LIC。