Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
Pediatr Blood Cancer. 2024 May;71(5):e30923. doi: 10.1002/pbc.30923. Epub 2024 Feb 22.
In pediatric transfusion-dependent thalassemia (TDT) patients, we evaluated the prevalence, pattern, and clinical associations of pancreatic siderosis and the changes in pancreatic iron levels and their association with baseline and changes in total body iron balance.
We considered 86 pediatric TDT patients consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network. Iron overload (IO) was quantified by R2* magnetic resonance imaging (MRI).
Sixty-three (73%) patients had pancreatic IO (R2* > 38 Hz). Global pancreas R2* values were significantly correlated with mean serum ferritin levels, MRI liver iron concentration (LIC) values, and global heart R2* values. Global pancreas R2* values were significantly higher in patients with altered versus normal glucose metabolism. Thirty-one patients also performed the follow-up MRI at 18 ± 3 months. Higher pancreatic R2* values were detected at the follow-up, but the difference versus the baseline MRI was not significant. The 20% of patients with baseline pancreatic IO showed no pancreatic IO at the follow-up. The 46% of patients without baseline pancreatic IO developed pancreatic siderosis. The changes in global pancreas R2* between the two MRIs were not correlated with baseline serum ferritin levels, baseline, final, and changes in MRI LIC values, or baseline pancreatic iron levels.
In children with TDT, pancreatic siderosis is a frequent finding associated with hepatic siderosis and represents a risk factor for myocardial siderosis and alterations of glucose metabolism. Iron removal from the pancreas is exceptionally challenging and independent from hepatic iron status.
在依赖输血的小儿地中海贫血症(TDT)患者中,我们评估了胰腺铁沉积的流行率、模式和临床相关性,以及胰腺铁水平的变化及其与总体铁平衡的基线和变化的相关性。
我们连续纳入了 86 例小儿 TDT 患者,这些患者均参与了地中海贫血症心肌铁过载网络的扩展研究。铁过载(IO)通过 R2*磁共振成像(MRI)进行量化。
63 例(73%)患者存在胰腺 IO(R2*>38 Hz)。整体胰腺 R2值与平均血清铁蛋白水平、MRI 肝脏铁浓度(LIC)值和整体心脏 R2值显著相关。与葡萄糖代谢正常的患者相比,胰腺 R2值在葡萄糖代谢异常的患者中显著更高。31 例患者还在 18±3 个月时进行了后续 MRI 检查。在随访时检测到胰腺 R2值更高,但与基线 MRI 相比无显著差异。20%基线存在胰腺 IO 的患者在随访时无胰腺 IO。46%无基线胰腺 IO 的患者发生了胰腺铁沉积。两次 MRI 之间的全球胰腺 R2*变化与基线血清铁蛋白水平、基线、最终和 MRI LIC 值变化或基线胰腺铁水平均无相关性。
在 TDT 儿童中,胰腺铁沉积是一种常见的发现,与肝脏铁沉积相关,是心肌铁沉积和葡萄糖代谢改变的危险因素。从胰腺中去除铁异常困难,并且与肝脏铁状态无关。