阵发性睡眠性血红蛋白尿症伴可逆性后部脑病综合征1例报道 Paroxysmal Nocturnal Hemoglobinuria with Posterior Reversible Encephalopathy Syndrome: A Case Report

Abstract

可逆性后部脑病综合征（posterior reversible encephalopathy syndrome，PRES）是一种以可逆性神经系统损害和特异性影像学表现为主的临床-影像学综合征，阵发性睡眠性血红蛋白尿症（paroxysmal nocturnal hemoglobinuria，PNH）是临床上的罕见疾病。本文介绍1例有PNH的老年男性患者，患者长期慢性溶血性贫血，不规律服用小剂量免疫抑制剂，短期间断输入共计10 U洗涤红细胞后，出现伴有头痛、痫性发作、精神行为异常、血压异常增高及微量蛛网膜下腔出血的PRES，此病需与脑静脉及静脉窦血栓形成和可逆性脑血管收缩综合征等疾病相鉴别。本文旨在提高临床医师对此类患者的预防、早期识别及应对能力。 Abstract: Posterior reversible encephalopathy syndrome (PRES) is a clinical-imaging syndrome dominated by reversible neurological damage and specific imaging manifestations, and paroxysmal nocturnal hemoglobinuria (PNH) is a clinically rare disease. This paper described a case of an elderly male patient with PNH who had long-term chronic hemolytic anemia. After irregularly taking low-dose immunosuppressants and short-term intermittent infusion of 10 U of washed erythrocytes, he developed a PRES with headache, seizures, mental behavioural abnormalities, abnormally high blood pressure, and trace subarachnoid hemorrhage. It should be differentiated from diseases such as cerebral venous and venous sinus thrombosis and reversible cerebral vasoconstriction syndrome. The aim of this paper is to improve the clinicians’ ability to prevent and identify this type of patients at an early stage.

Keywords

• 阵发性睡眠性血红蛋白尿症
• 可逆性后部脑病综合征
• 输血
• 蛛网膜下腔出血
• 痫性发作
• paroxysmal nocturnal hemoglobinuria
• posterior reversible encephalopathy syndrome
• blood transfusion
• subarachnoid hemorrhage
• seizure