Meloni Antonella, Pistoia Laura, Ricchi Paolo, Longo Filomena, Cecinati Valerio, Sorrentino Francesco, Borsellino Zelia, Bagnato Sergio, Rossi Vincenza, Fina Priscilla, Riva Ada, Renne Stefania, Peritore Giuseppe, Positano Vincenzo, Cademartiri Filippo
Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy.
J Clin Med. 2024 Aug 14;13(16):4791. doi: 10.3390/jcm13164791.
: This multicenter, retrospective, population-based, matched-cohort study compared clinical characteristics and magnetic resonance imaging (MRI) findings, including hepatic, pancreatic, and cardiac iron levels and cardiac function, between 135 adult regularly transfused thalassemia intermedia (TI) patients (44.73 ± 12.16 years, 77 females) and 135 age- and sex-matched thalassemia major (TM) patients (43.35 ± 9.83 years, 77 females), enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. The MRI protocol included the quantification of hepatic, pancreatic, and cardiac iron levels (R2* technique), the assessment of biventricular function parameters (cine images), and the detection of replacement myocardial fibrosis (late gadolinium enhancement technique). Age, sex, frequency of splenectomy and chelation, and serum ferritin levels were not significantly different ( > 0.05) between the two groups, but TI patients started regular transfusions significantly later ( < 0.0001) and showed significantly lower pre-transfusion hemoglobin levels ( = 0.005). No difference was found in hepatic iron levels ( = 0.853). TI patients exhibited significantly lower pancreatic R2* values ( < 0.0001), also correcting for the duration of regular transfusions, and significantly lower cardiac R2* values ( < 0.0001). In the receiver operating characteristic analysis, pancreatic iron was the strongest discriminator between the two diseases. Left and right ventricular end-diastolic volume indexes were significantly higher in TI than in TM patients ( = 0.003 and = 0.046, respectively), but the correction for the duration of regular transfusions removed the disease-specific differences ( > 0.05). Left ventricular (LV) mass index was significantly higher in TI ( = 0.049), while no difference ( > 0.05) was found in biventricular ejection fractions and replacement myocardial fibrosis. TI patients showed lower pancreatic and cardiac iron burden and more pronounced LV hypertrophy. These differences could not be explained by the different duration of the transfusional regimen.
这项多中心、回顾性、基于人群的配对队列研究，比较了135例成年中间型地中海贫血（TI）定期输血患者（44.73±12.16岁，77名女性）与135例年龄和性别匹配的重型地中海贫血（TM）患者（43.35±9.83岁，77名女性）的临床特征和磁共振成像（MRI）结果，这些患者均纳入地中海贫血网络的心肌铁过载扩展研究。MRI方案包括肝、胰、心铁水平的定量（R2技术）、双心室功能参数的评估（电影图像）以及替代性心肌纤维化的检测（延迟钆增强技术）。两组之间的年龄、性别、脾切除和螯合频率以及血清铁蛋白水平无显著差异（>0.05），但TI患者开始定期输血的时间明显更晚（<0.0001），且输血前血红蛋白水平显著更低（=0.005）。肝铁水平无差异（=0.853）。TI患者的胰腺R2值显著更低（<0.0001），校正定期输血持续时间后也是如此，心脏R2*值也显著更低（<0.0001）。在受试者工作特征分析中，胰腺铁是区分这两种疾病的最强指标。TI患者的左、右心室舒张末期容积指数显著高于TM患者（分别为=0.003和=0.046），但校正定期输血持续时间后消除了疾病特异性差异（>0.05）。TI患者的左心室（LV）质量指数显著更高（=0.049），而双心室射血分数和替代性心肌纤维化无差异（>0.05）。TI患者的胰腺和心脏铁负荷较低，左心室肥厚更明显。这些差异无法用输血方案的不同持续时间来解释。
