Mazza P, Giua R, De Marco S, Bonetti M G, Amurri B, Masi C, Lazzari G, Rizzo C, Cervellera M, Peluso A
Servizio di Ematologia, Ospedale SS Annunziata, Taranto, Italy.
Haematologica. 1995 Sep-Oct;80(5):398-404.
Iron overload in patients with thalassemia is a common feature which requires continuous chelation therapy and monitoring. Serum ferritin determination is widely accepted as a simple method for following iron load in patients with primary hemochromatosis; however, several reports on thalassemic patients emphasize that ferritinemia is not accurate and that other methods such as direct measurement of iron in the liver (HIC) and magnetic resonance imaging (MRI) are more precise.
In order to contribute to the general understanding of iron load in thalassemia we used liver MRI to study 33 thalassemic patients, most of whom were also evaluated for iron content by liver biopsy. The data were then compared with serum ferritin levels.
Ferritin levels ranged between 276 and 8031 ng/mL, and liver iron content ranged from 1.6 to 31.0 mg/g dry weight; grade III or IV liver siderosis was recorded in 23/33 patients, just as 23/33 patients were found to have severe or very severe siderosis at MRI. Significant correlations with ferritin levels were recorded between grade IV and grades III, II and I (p < 0.01, p = 0.02, and p = 0.03, respectively). Ferritinemia also showed significant linearity with liver iron content (r = 0.603, p = 0.001). No significant differences of levels were recorded, however, between patients found to have severe and those with mild iron load at MRI (p = 0.073).
Our study shows that serum ferritin levels exhibit a tendency to be significantly correlated with the true status of hemochromatosis in thalassemic patients; however, the discrepancies recorded in several patients and the scarce or total lack of correlation with MRI suggest exploring other approaches to this problem in order to make proper decisions about therapy.
地中海贫血患者的铁过载是一个常见特征,需要持续的螯合治疗和监测。血清铁蛋白测定被广泛认为是一种用于跟踪原发性血色素沉着症患者铁负荷的简单方法;然而,一些关于地中海贫血患者的报告强调,血清铁蛋白水平并不准确,而其他方法,如肝脏铁直接测量(HIC)和磁共振成像(MRI)则更为精确。
为了促进对地中海贫血中铁负荷的总体理解,我们使用肝脏MRI研究了33例地中海贫血患者,其中大多数患者还通过肝脏活检评估了铁含量。然后将数据与血清铁蛋白水平进行比较。
铁蛋白水平在276至8031 ng/mL之间,肝脏铁含量在1.6至31.0 mg/g干重之间;23/33例患者记录为III级或IV级肝脏铁沉积,同样在MRI检查中发现23/33例患者有严重或非常严重的铁沉积。IV级与III级、II级和I级之间记录到与铁蛋白水平有显著相关性(分别为p < 0.01、p = 0.02和p = 0.03)。血清铁蛋白水平也与肝脏铁含量呈显著线性关系(r = 0.603,p = 0.001)。然而,在MRI检查中发现铁负荷严重的患者与轻度铁负荷患者之间,水平没有显著差异(p = 0.073)。
我们的研究表明,血清铁蛋白水平与地中海贫血患者血色素沉着症的真实状况呈显著相关趋势;然而,在一些患者中记录到的差异以及与MRI缺乏或完全缺乏相关性,提示应探索解决该问题的其他方法,以便对治疗做出恰当决策。