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输血依赖型地中海贫血患者“正常”血清铁蛋白水平的设定:我们目前的策略

Setting for "Normal" Serum Ferritin Levels in Patients with Transfusion-Dependent Thalassemia: Our Current Strategy.

作者信息

Spasiano Anna, Meloni Antonella, Costantini Silvia, Quaia Emilio, Cademartiri Filippo, Cinque Patrizia, Pepe Alessia, Ricchi Paolo

机构信息

Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", 80131 Napoli, Italy.

Cardiovascular and Neuroradiological Multimodality Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.

出版信息

J Clin Med. 2021 Dec 20;10(24):5985. doi: 10.3390/jcm10245985.

Abstract

This cross-sectional study aimed to establish the association between serum ferritin levels and organ iron overload (IO) and overall morbidity in transfusion-dependent thalassemia (TDT) patients. One hundred and three TDT patients (40.03 ± 9.15 years; 57.3% females) with serum ferritin < 2500 ng/mL were included. IO was assessed by T2* magnetic resonance imaging. Three groups were identified based on mean serum ferritin levels: <500 ng/mL (group 0; N = 32), 500-1000 ng/mL (group 1; N = 43), and 1000-2500 ng/mL (group 2; N = 28). All demographic and biochemical parameters were comparable among the three groups, with the exception of the triglycerides being significantly lower in group 0 than in group 2. No difference was found in the frequency of hepatic, endocrine, and cardiac complications. Hepatic IO was significantly less frequent in group 0 versus both groups 1 and 2. No patient with a serum ferritin level < 500 ng/mL had significant myocardial IO and alterations in the main hematological parameters. No difference in the distribution of the different chelation regimens was found. Serum ferritin < 500 ng/mL appears to be achievable and safe for several TDT patients. This target is associated with the absence of significant cardiac iron and significantly lower hepatic IO and triglycerides that are well-demonstrated markers for cardiac and liver complications.

摘要

这项横断面研究旨在确定输血依赖型地中海贫血(TDT)患者血清铁蛋白水平与器官铁过载(IO)及总体发病率之间的关联。纳入了103例血清铁蛋白<2500 ng/mL的TDT患者(年龄40.03±9.15岁;女性占57.3%)。通过T2*磁共振成像评估IO。根据平均血清铁蛋白水平分为三组:<500 ng/mL(0组;N = 32)、500 - 1000 ng/mL(1组;N = 43)和1000 - 2500 ng/mL(2组;N = 28)。三组之间所有人口统计学和生化参数均具有可比性,但0组甘油三酯显著低于2组。肝、内分泌和心脏并发症的发生率未见差异。0组肝IO的发生率显著低于1组和2组。血清铁蛋白水平<500 ng/mL的患者均无显著心肌IO及主要血液学参数改变。不同螯合方案的分布无差异。血清铁蛋白<500 ng/mL对部分TDT患者似乎是可实现且安全的。该目标与无显著心脏铁、显著降低的肝IO以及甘油三酯水平相关,而甘油三酯是心脏和肝脏并发症的良好指标。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/710c/8708030/af7dccdb5245/jcm-10-05985-g001.jpg

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