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骨髓增生异常综合征患者前瞻性心脏磁共振成像调查:来自意大利网络的见解。

Prospective cardiac magnetic resonance imaging survey in myelodysplastic syndrome patients: insights from an Italian network.

机构信息

MRI Unit, Fondazione G. Monasterio CNR-Regione Toscana, Via Moruzzi, 1, 56124, Pisa, Italy.

Reparto di Ematologia, Azienda Sanitaria Provinciale Caltanissetta-Ospedale "Sant'Elia", Caltanisetta, Italy.

出版信息

Ann Hematol. 2021 May;100(5):1139-1147. doi: 10.1007/s00277-021-04495-y. Epub 2021 Mar 19.

Abstract

We prospectively evaluated changes in cardiac and hepatic iron overload (IO) and in morpho-functional cardiac parameters and myocardial fibrosis by magnetic resonance imaging (MRI) in patients with low-risk and intermediate-1-risk myelodysplastic syndromes (MDS). Fifty patients enrolled in the Myocardial Iron Overload in MyElodysplastic Diseases (MIOMED) study were followed for 12 months. IO was quantified by the T2* technique and biventricular function parameters by cine images. Macroscopic myocardial fibrosis was detected by late gadolinium enhancement technique. Twenty-eight patients (71.89±8.46 years; 8 females) performed baseline and follow-up MRIs. Thirteen patients had baseline hepatic IO, with a higher frequency among transfusion-dependent patients. Out of the 15 patients with a baseline MRI liver iron concentration <3 mg/g/dw, two (non-chelated) developed hepatic IO. Thirteen (46.4%) patients had an abnormal T2* value in at least one myocardial segment. One patient without hepatic IO and non-transfused had baseline global T2* <20 ms. Among the 15 patients with no baseline myocardial IO (MIO), 2 worsened. There was a significant increase in both left and right ventricular end-diastolic volume indexes. Thirty-six percent of patients showed myocardial fibrosis correlating with aging. Two new occurrences were detected at the follow-up. In conclusion, by a more sensitive segmental approach, MIO is quite frequent in MDS patients and it can be present also in non-transfused patients and in absence of detectable hepatic iron. The incidence of cardiac and hepatic IO and of myocardial fibrosis and the increase in biventricular volumes after a 12-month interval suggest performing periodic MRI scans to better manage MDS patients.

摘要

我们前瞻性地评估了低危和中危 1 级骨髓增生异常综合征(MDS)患者心脏和肝脏铁过载(IO)以及心脏形态和功能参数和心肌纤维化的变化。50 名患者入组了 Myocardial Iron Overload in MyElodysplastic Diseases(MIOMED)研究,并随访了 12 个月。通过 T2技术量化 IO,通过电影图像量化双心室功能参数。通过延迟钆增强技术检测宏观心肌纤维化。28 名患者(71.89±8.46 岁;8 名女性)进行了基线和随访 MRI。13 名患者存在基线肝 IO,其中依赖输血的患者更为常见。在基线 MRI 肝铁浓度<3mg/g/dw 的 15 名患者中,有 2 名(未螯合)发生肝 IO。13(46.4%)名患者至少一个心肌节段的 T2值异常。1 名无肝 IO 且未输血的患者基线整体 T2*<20ms。在 15 名无基线心肌 IO(MIO)的患者中,2 名恶化。左、右心室舒张末期容积指数均显著增加。36%的患者出现与年龄相关的心肌纤维化。在随访中检测到 2 例新发病例。总之,通过更敏感的节段性方法,MDS 患者的 MIO 相当常见,即使在未输血的患者和无可检测到的肝铁的情况下也可能存在。12 个月后心脏和肝脏 IO、心肌纤维化以及双心室容积的增加的发生率表明需要定期进行 MRI 扫描,以更好地管理 MDS 患者。

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