阵发性睡眠性血红蛋白尿合并脑静脉窦血栓形成1例并文献复习

Paroxysmal nocturnal hemoglobinuria with cerebral venous sinus thrombosis：a case report and literature review

阵发性睡眠性血红蛋白尿（paroxysmalnocturnalhemoglobinuria,PNH）是一种后天获得性造血干细胞克隆缺陷性疾病，可表现为溶血性贫血、血红蛋白尿和静脉血栓形成。脑静脉窦血栓形成（cerebral venous sinus thrombosis, CVST ）是PNH的一种少见但较严重的并发症。文章报道1例PNH合并CVST患者，并对相关文献进行了复习。对于伴有CVST 危险因素且表现为头痛、颅内压升高等神经系统症状的患者，应及早行颅脑影像学检查明确诊断，积极给予抗凝、脱水、祛除病因、控制并发症等方面的治疗。

Paroxysmalnocturnalhemoglobinuria（PNH）isadefectdiseaseofacquiredclonal hematopoietic stem cel s. It can be expressed as hemolytic anemia, hemoglobinuria, and venous thrombosis. Cerebral venous sinus thrombosis （CVST） is a rare but serious complication of PNH. Here we report a PNH patient with CVST and reviewthe relevant literature. For patients who have the risk factors for CVST and neurological symptoms, such as headache and increased intracranial pressure, should early conduct brain imaging examination and make the diagnosis clear, and give an active treatment in the aspects of anticoagulation, dehydration, eliminating the causes of disease, and controling complications.