阵发性睡眠性血红蛋白尿并发颅内静脉窦血栓形成一例

Paroxysmal nocturnal hemoglobinuria complicated with cerebral venous sinus thrombosis:a case report

颅内静脉窦血栓形成(CVST)是一种相对少见的特殊类型脑血管病,是指患者颅内静脉窦或静脉出现血栓和闭塞,其病因不同且临床表现缺乏特异性,因此容易出现漏诊、误诊。作者报道了1例以头痛起病,阵发性睡眠性血红蛋白尿(PNH)并发CVST的患者,结合国内外文献进行了分析,探讨了CVST的发病机制、治疗方法及如何获得更好的预后,以期提高临床医师对PNH并发CVST的认识。

Cerebral venous sinus thrombosis(CVST)is a relatively rare and special type of cerebrovascular disease,which refers to thrombosis and occlusion of intracranial venous sinus or vein.Due to different causes and lack of specificity in clinical manifestations,it is easy to miss diagnosis and misdiagnosis.The authors reported a case of headache onset,paroxysmal nocturnal hemoglobinuria(PNH)complicated with CVST,and analyzed domestic and foreign literatures,to explore the pathogenesis,treatment and better prognosis of CVST,and to improve clinicians′understanding of PNH complicated with CVST.