Magnetic resonance imaging to determine the incidence of brain ischaemia in patients with β-thalassaemia intermedia
Magnetic resonance imaging to determine the incidence of brain ischaemia in patients with β-thalassaemia intermedia
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DOI：
10.1160/th09-09-0661
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发表时间：
2010-05-01
影响因子：
6.7
通讯作者：
Rachmilewitz, Eliezer A.
中科院分区：
文献类型：
--
作者：
Karimi, Mehran;Bagheri, Hadi;Rachmilewitz, Eliezer A.
长期生存的-地中海贫血患者的一个并发症是慢性高凝状态，这导致包括大脑在内的主要器官发生血栓栓塞事件。我们测定了一个接受脾切除术的无症状中-地中海贫血（TI）患者脑内微血栓形成的发生率。本回顾性研究包括200例根据血球计数、血红蛋白（Hb）电泳和临床资料诊断的TI患者。年龄18 ~ 34岁，女性19例（63.3%），男性11例（36.7%）。我们随机选择了30例符合纳入标准的患者：Hb浓度bbb70 g/dl)，脾切除术和血小板计数> 50万/ml。平均Hb浓度为8.4 g/dl，平均铁蛋白浓度为519 ng/ml。每位患者都进行了核磁共振成像（MRI），并由神经放射专家对结果进行了解释。影像学检查显示28%的患者有病理表现。6例脑白质改变提示缺血，2例有小梗死的迹象。结论：1)在这一小部分患者中，可考虑在20岁时进行诊断性磁共振成像监测早期无症状或亚临床脑血管损伤，并每3-5年重复一次。2)无症状脑缺血患者建议使用抗血小板聚集剂治疗。3)这些结果需要在更大的其他类型的地中海贫血患者中得到证实，这些患者是多次输血或脾脏完好的。
One complication in patients with beta-thalassaemia who had prolonged survival is chronic hypercoagulable state, which results in thromboembolic events involving major organs including the brain. We determined the prevalence of microthrombosis in the brain in an asymptomatic subgroup of patients with beta-thalassaemia intermedia (TI) who had undergone splenectomy. This retrospective review included 200 patients with TI diagnosed on the basis of blood count, haemoglobin (Hb) electrophoresis and clinical data. Their ages ranged from 18 to 34 years, 19 (63.3%) were women and 11(36.7%) were men. We selected 30 patients at random who fulfilled the inclusion criteria: Hb concentration > 7 g/dl), splenectomy and platelet count > 500,000/ml. Their mean Hb concentration was 8.4 g/dl and their mean ferritin concentration was 519 ng/ml. Magnetic resonance imaging (MRI) was done in every patient, and the findings were interpreted by an expert neuroradiologist. Imaging studies showed pathological findings in 28% of the patients. Six had changes in the white matter suggestive of ischaemia and two had evidence of small infarctions. Conclusion: 1) In this small subset of patients diagnostic magnetic resonance imaging to monitor early asymptomatic or subclinical vascular damage in the brain can be considered when they reach the age of 20 years, and repeated every 3-5 years. 2) Treatment with antiplatelet aggregants is suggested in patients with documented asymptomatic brain ischaemia. 3) These results require confirmation in a larger group of similar patients with other types of thalassaemia who are multitransfused or have an intact spleen.