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成人无症状β-地中海贫血患者的脑血管病变无增加。一项多中心多模态磁共振研究。

No evidence of increased cerebrovascular involvement in adult neurologically-asymptomatic β-Thalassaemia. A multicentre multimodal magnetic resonance study.

机构信息

Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Università degli studi della Campania "Luigi Vanvitelli", Napoli, Italy.

Neuroradiologia, Università degli Studi di Napoli "Federico II", Napoli, Italy.

出版信息

Br J Haematol. 2019 May;185(4):733-742. doi: 10.1111/bjh.15834. Epub 2019 Mar 5.

DOI:10.1111/bjh.15834
PMID:30836432
Abstract

Multi-factorial causes jeopardize brain integrity in β-thalassaemia. Intracranial parenchymal and vascular changes have been reported among young β-thalassaemia patients but conventional magnetic resonance imaging (MRI) findings are contradictory making early MRI and magnetic resonance angiography (MRA)/venography monitoring a matter of debate. This study prospectively investigated 75 neurologically asymptomatic β-thalassaemia patients (mean-age 35·2 ± 10·7 years; 52/75 transfusion-dependent; 41/75 splenectomised) using a 3T magnetic resonance scanner; clinical, laboratory and treatment data were also collected. White matter ischaemic-like abnormalities, intracranial artery stenoses, aneurysms and sinus venous thrombosis were compared between patients and 56 healthy controls (mean-age 33·9 ± 10·8 years). No patient or control showed silent territorial or lacunar strokes, intracranial artery stenoses or signs of sinus thrombosis. White matter lesions were found both in patients (35/75, 46·7%) and controls (28/56, 50·0%), without differences in terms of number (4·0 ± 10·6 vs. 4·6 ± 9·1, P = 0·63), size and Fazekas' Score. Intracranial aneurysms did not differ between patients and controls for incidence rate (7/75, 9·3% vs. 5/56, 8·9%), size and site. Vascular and parenchymal abnormality rate did not differ according to treatments or clinical phenotype. According to this study, asymptomatic β-thalassaemia patients treated according to current guidelines do not seem to carry an increased risk of brain and intracranial vascular changes, thus weakening recommendations for regular brain MRI monitoring.

摘要

多种因素导致β-地中海贫血患者脑损伤。已有报道称,年轻的β-地中海贫血患者存在颅内实质和血管变化,但常规磁共振成像(MRI)结果存在矛盾,使得早期 MRI 和磁共振血管造影(MRA)/静脉造影监测成为争议话题。本研究前瞻性调查了 75 例神经无症状β-地中海贫血患者(平均年龄 35.2±10.7 岁;52/75 例依赖输血;41/75 例脾切除术),使用 3T 磁共振扫描仪;还收集了临床、实验室和治疗数据。比较了患者和 56 名健康对照者(平均年龄 33.9±10.8 岁)之间的脑白质缺血样异常、颅内动脉狭窄、动脉瘤和静脉窦血栓形成。无患者或对照者出现无症状性区域性或腔隙性脑卒中、颅内动脉狭窄或静脉窦血栓形成迹象。患者(35/75,46.7%)和对照者(28/56,50.0%)均存在脑白质病变,但在病变数量(4.0±10.6 与 4.6±9.1,P=0.63)、大小和 Fazekas 评分方面无差异。颅内动脉瘤的发生率(7/75,9.3% vs. 5/56,8.9%)、大小和部位在患者和对照者之间无差异。血管和实质异常发生率与治疗或临床表型无关。根据本研究,按照现行指南治疗的无症状β-地中海贫血患者似乎没有增加脑和颅内血管变化的风险,因此削弱了定期脑 MRI 监测的建议。

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