Vitrano Angela, Calvaruso Giuseppina, Tesé Lorenzo, Gioia Francesco, Cassarà Filippo, Campisi Saveria, Butera Franco, Commendatore Valeria, Rizzo Michele, Santoro Vincenzo, Cigna Valeria, Quota Alessandra, Bagnato Sabrina, Argento Crocetta, Fidone Carmelo, Schembari Dario, Gerardi Calogera, Barbiera Filippo, Bellisssima Giuseppe, Giugno Giovanni, Polizzi Gesualdo, Rosso Rosamaria, Abbate Giovanna, Caruso Vincenzo, Chiodi Elisabetta, Gamberini Maria Rita, Giorgi Benedetta, Putti Maria Caterina, Filosa Aldo, De Ritis Maria Rosaria, Oliva Esther, Arcadi Nicola, Fustaneo Maria, Mistretta Laura, Di Maggio Rosario, Sacco Massimiliano, Veronica Di Salvo, Giangreco Antonino, Maggio Aurelio
Campus of Haematology Franco e Piera Cutino- A.O.O.R. 'Villa Sofia-Cervello', Palermo, Italy.
Ospedale Umberto I, Siracusa, Italy.
Eur J Haematol. 2016 Oct;97(4):361-70. doi: 10.1111/ejh.12740. Epub 2016 Feb 23.
Real-life data on the use of R2 MRI for the assessment of liver iron concentration (LIC) remain limited.
We conducted a cross-sectional analysis on 363 patients (mean age 35.6 yr, 44.1% men) with hemoglobinopathies (204 β-thalassemia major [TM], 102 β-thalassemia intermedia [TI], and 57 sickle cell disease [SCD]) that were evaluated with R2 MRI as part of LICNET, an MRI network of 13 Italian treatment centers.
The mean LIC was 7.8 mg/g (median: 4.0), with high LIC (>7 mg/g) noted in both transfused (TM, TI 37%; SCD 38%) and non-transfused (TI 20%) patients. Ferritin levels correlated with LIC in both transfused (TM, TI, SCD) and non-transfused (TI) patients (P < 0.001), although lower values predicted high LIC in non-transfused patients (1900 vs. 650 ng/mL in TM vs. non-transfused TI). A correlation between LIC and ALT levels was only noted in HCV-negative patients (rs = 0.316, P < 0.001). The proportion of patients with high LIC was significantly different between iron chelators used (P = 0.023), with the lowest proportion in deferasirox (30%) and highest in deferiprone (53%)-treated patients.
High LIC values persist in subgroups of patients with hemoglobinopathy, warranting closer monitoring and management optimization, even for non-transfused patients with relatively low ferritin levels.
关于使用R2磁共振成像（MRI）评估肝脏铁浓度（LIC）的实际应用数据仍然有限。
我们对363例血红蛋白病患者（平均年龄35.6岁，44.1%为男性）进行了横断面分析，这些患者包括204例重型β地中海贫血（TM）、102例中间型β地中海贫血（TI）和57例镰状细胞病（SCD），他们作为LICNET（一个由13个意大利治疗中心组成的MRI网络）的一部分接受了R2 MRI评估。
平均LIC为7.8 mg/g（中位数：4.0），在接受输血的患者（TM、TI为37%；SCD为38%）和未输血的患者（TI为20%）中均发现高LIC（>7 mg/g）。在接受输血的患者（TM、TI、SCD）和未输血的患者（TI）中，铁蛋白水平均与LIC相关（P < 0.001），尽管在未输血的患者中较低的铁蛋白水平预示着高LIC（TM中为1900 ng/mL，未输血的TI中为650 ng/mL）。仅在丙型肝炎病毒（HCV）阴性的患者中发现LIC与丙氨酸转氨酶（ALT）水平之间存在相关性（rs = 0.316，P < 0.001）。使用不同铁螯合剂治疗的患者中高LIC患者的比例存在显著差异（P = 0.023），地拉罗司治疗的患者中比例最低（30%），去铁酮治疗的患者中比例最高（53%）。
血红蛋白病患者亚组中持续存在高LIC值，即使对于铁蛋白水平相对较低的未输血患者，也需要更密切的监测和优化管理。
