Iron overload in non-transfusion-dependent thalassemia- experience at a tertiary care center in India

Sujata Sharma, Lekha Parikh, Prachi Pandhare
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Abstract

Background

Non-transfusion-dependent thalassemia (NTDT) patients can be at risk of iron overload, despite not being transfused frequently. Iron overload is a major risk factor that increases morbidity and mortality in non-transfusion dependent thalassemia patients.

Aim

This study looked at the prevalence of iron overload in NTDT patients and evaluated the relationship between serum ferritin and calculated liver iron concentration (LIC) based on T2∗MRI.

Methods

We conducted an observational and retrospective study in a tertiary care centre of the metropolitan country of India from January 2019 to November 2020. We evaluated the prevalence of iron overload in NTDT patients, the relationship between number of PRC transfusions, serum ferritin levels, hepatic and cardiac iron as measured by validated R2 magnetic resonance imaging with T2∗ MRI values.

Results & discussion

Total of 56 NTDT patients were 8–12 years old with a mean age of 9.38 years. Majority of our study participants were beta thalassemia intermedia 45 (80.4 %) followed by HbE/β thalassemia 7(12.5 %) and 2 each (3.6 %) as HbH disease and Delta Beta Thalassemia. Magnetic resonance Imaging (T2∗ MRI) was done in 56 patients. Of these 35(62.5 %) had hepatic overload and 9 (16.1 %)) had cardiac overload. The mean serum ferritin level was 556.93 ng/ml. There was a negative correlation between the number of PRC transfusion, and liver T2∗ MRI values (r = −0.209, P = 0.123) as well as cardiac T2∗MRI T2∗ (r = −0.231, P = 0.087). Since T2∗ MRI values are inversely proportional to iron overload, the higher the number of PRC transfusions, the lower were the T2∗ values. We found that liver iron overload was present in half of the participants (n = 17, 53.13 %) even those who were never transfused or received less than 10 units.

Conclusion

Our study shows that there is a high prevalence of liver iron overload in NTDT patients. Serum Ferritin is an unreliable indicator, whereas T2∗ MRI values are better for assessing iron overload in NTDT patients.
非输血依赖型地中海贫血的铁超载——印度三级保健中心的经验
背景:非输血依赖型地中海贫血(NTDT)患者尽管不经常输血,但可能存在铁超载的风险。铁超载是增加非输血依赖型地中海贫血患者发病率和死亡率的一个主要危险因素。目的研究NTDT患者铁超载的发生率,并评估血清铁蛋白与基于T2 * MRI计算的肝铁浓度(LIC)之间的关系。方法我们于2019年1月至2020年11月在印度大都市国家的一家三级保健中心进行了一项观察性和回顾性研究。我们评估了NTDT患者铁超载的患病率,以及PRC输注次数、血清铁蛋白水平、肝脏和心脏铁之间的关系(通过验证的R2磁共振成像与T2 * MRI值测量)。结果,56例NTDT患者年龄8 ~ 12岁,平均年龄9.38岁。我们的大多数研究参与者是β -地中海贫血中间45(80.4%),其次是HbE/β -地中海贫血7(12.5%)和2(3.6%),分别为HbH病和β -地中海贫血。对56例患者进行磁共振成像(T2 * MRI)检查。其中35例(62.5%)有肝负荷过重,9例(16.1%)有心脏负荷过重。血清铁蛋白平均水平为556.93 ng/ml。输血次数与肝脏T2 * MRI值(r = - 0.209, P = 0.123)和心脏T2 * MRI T2 *值(r = - 0.231, P = 0.087)呈负相关。由于T2 * MRI值与铁负荷成反比,因此PRC输注次数越多,T2 *值越低。我们发现一半的参与者(n = 17, 53.13%)存在肝铁超载,即使是那些从未输血或输血量少于10单位的参与者。结论我们的研究表明,NTDT患者存在高发生率的肝铁超载。血清铁蛋白是一个不可靠的指标,而T2 * MRI值更好地评估NTDT患者的铁超载。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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