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磁共振成像确定中间型β地中海贫血患者脑缺血的发生率。

Magnetic resonance imaging to determine the incidence of brain ischaemia in patients with beta-thalassaemia intermedia.

机构信息

Hematology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

Thromb Haemost. 2010 May;103(5):989-93. doi: 10.1160/TH09-09-0661. Epub 2010 Feb 19.

Abstract

UNLABELLED

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 b-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.
摘要

目的

研究在接受脾切除术的β-地中海贫血中间型(TI)无症状亚组患者中,脑内微血栓形成的发生率。

方法

本回顾性研究纳入了 200 例基于血常规、血红蛋白电泳和临床数据诊断为 TI 的患者。他们的年龄为 18 至 34 岁,其中 19 例(63.3%)为女性,11 例(36.7%)为男性。我们随机选择了 30 名符合纳入标准的患者:血红蛋白浓度>7 g/dl)、脾切除术和血小板计数>500,000/ml。他们的平均血红蛋白浓度为 8.4 g/dl,平均铁蛋白浓度为 519 ng/ml。每位患者均进行了磁共振成像(MRI)检查,由神经放射学专家解读结果。影像学研究显示,28%的患者存在病理学发现。6 例患者的白质有缺血改变,2 例有小梗死证据。

结论

1)在这一小部分患者中,当他们达到 20 岁时,可以考虑进行诊断性磁共振成像以监测早期无症状或亚临床的脑血管损伤,并每 3-5 年重复一次。2)建议对有记录的无症状脑缺血患者使用抗血小板聚集剂进行治疗。3)这些结果需要在其他接受多次输血或保留脾脏的具有不同类型地中海贫血的类似患者群体中进一步确认。

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