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本文引用的文献

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Eculizumab for treating patients with paroxysmal nocturnal hemoglobinuria.依库珠单抗用于治疗阵发性夜间血红蛋白尿患者。
Cochrane Database Syst Rev. 2014 Oct 30(10):CD010340. doi: 10.1002/14651858.CD010340.pub2.
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Paroxysmal nocturnal hemoglobinuria.阵发性睡眠性血红蛋白尿症。
Blood. 2014 Oct 30;124(18):2804-11. doi: 10.1182/blood-2014-02-522128. Epub 2014 Sep 18.
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Long-term safety and efficacy of sustained eculizumab treatment in patients with paroxysmal nocturnal haemoglobinuria.阵发性睡眠性血红蛋白尿症患者持续依库珠单抗治疗的长期安全性和疗效。
Br J Haematol. 2013 Jul;162(1):62-73. doi: 10.1111/bjh.12347. Epub 2013 Apr 25.
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Thrombosis in paroxysmal nocturnal hemoglobinuria.阵发性睡眠性血红蛋白尿症中的血栓形成。
Blood. 2013 Jun 20;121(25):4985-96; quiz 5105. doi: 10.1182/blood-2012-09-311381. Epub 2013 Apr 22.
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Mechanisms and clinical implications of thrombosis in paroxysmal nocturnal hemoglobinuria.阵发性睡眠性血红蛋白尿症中血栓形成的机制及临床意义。
J Thromb Haemost. 2012 Jan;10(1):1-10. doi: 10.1111/j.1538-7836.2011.04562.x.
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Paroxysmal nocturnal hemoglobinuria from bench to bedside.阵发性夜间血红蛋白尿从基础到临床。
Clin Transl Sci. 2011 Jun;4(3):219-24. doi: 10.1111/j.1752-8062.2011.00262.x.
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Aetiological diagnosis of ischaemic stroke in young adults.青年缺血性脑卒中的病因诊断。
Lancet Neurol. 2010 Nov;9(11):1085-96. doi: 10.1016/S1474-4422(10)70251-9.
8
Epidemiology of rare anaemias in Europe.欧洲罕见贫血症的流行病学。
Adv Exp Med Biol. 2010;686:375-96. doi: 10.1007/978-90-481-9485-8_22.
9
Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria.用于治疗阵发性夜间血红蛋白尿的补体抑制剂依库珠单抗的发现与研发。
Nat Biotechnol. 2007 Nov;25(11):1256-64. doi: 10.1038/nbt1344.
10
Thrombosis in paroxysmal nocturnal hemoglobinuria: sites, risks, outcome. An overview.阵发性夜间血红蛋白尿中的血栓形成:部位、风险、结局。综述
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阵发性夜间血红蛋白尿背景下的复发性脑缺血事件

Recurrent cerebral ischaemic events in the setting of paroxysmal nocturnal haemoglobinuria.

作者信息

Azevedo Luísa, Costa Mariana Reis, Fonseca Ana Catarina, Pinho e Melo Teresa

机构信息

Department of Neurosciences and Mental Health, Stroke Unit, Lisboa, Portugal.

出版信息

BMJ Case Rep. 2016 Feb 4;2016:bcr2015213603. doi: 10.1136/bcr-2015-213603.

DOI:10.1136/bcr-2015-213603
PMID:26847804
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4746544/
Abstract

The authors present a case of a 28-year-old man with a known history of paroxysmal nocturnal haemoglobinuria (PNH), under platelet antiaggregation, admitted following recurrent transitory arterial ischaemic attacks. Concomitant thrombosis of the superior mesenteric artery and bilateral renal infarction was found. Cardioembolism, namely patent foramen ovale, was excluded and anticoagulation added, with no further events on 10-month follow-up. PNH is a rare acquired disorder of haematopoietic stem cells, characterised by haemolytic anaemia, pancytopenia and thrombotic events classically involving the venous system. Reports of cerebral artery stroke and accompanying intra-abdominal arterial thrombosis are especially rare. Complementary investigation and treatment options in these patients are discussed.

摘要

作者报告了一例28岁男性患者,该患者有阵发性夜间血红蛋白尿(PNH)病史,正在接受血小板抗聚集治疗,因反复短暂性动脉缺血性发作入院。检查发现伴有肠系膜上动脉血栓形成和双侧肾梗死。排除了心源性栓塞,即卵圆孔未闭,并加用了抗凝治疗,在10个月的随访中未再发生其他事件。PNH是一种罕见的造血干细胞获得性疾病,其特征为溶血性贫血、全血细胞减少和血栓形成事件,典型情况累及静脉系统。脑动脉卒中及伴有腹内动脉血栓形成的报告尤为罕见。本文讨论了这些患者的辅助检查及治疗选择。