摘要
目的:提高对造血干细胞移植(HSCT)术后不常见的神经系统并发症-韦尼克脑病(WE)的临床表现、诊断和治疗的认识及掌握。方法:回顾分析自2016年1月至2017年1月在航天中心医院血液科接受HSCT治疗,术后诊断WE的4例病例的临床特点,诊疗经过以及预后随访。结果:4例确诊WE病例约占同期移植患者的3%,其中男性3例,女性1例,年龄为8-20岁,2例AML,2例ALL。4例中仅1例具有典型的眼颤,共济失调和全脑功能障碍的三联征,所有病例均早期表现为神志模糊和语言障碍。头颅CT平扫均阴性,无提示意义。头颅MRI检查显示,3例具有典型部位(丘脑、第四脑室底、第三脑室和中脑导水管)结构异常病变,另1例仅在乳头体发现病变。全部病例确诊后均接受每日100 mg的维生素B_1肌内注射治疗,分别于治疗后2 d、不详、3 d、4 d后症状改善,2周内全部病例神经系统症状获得完全缓解,随访7-12个月后均未见WE复发。结论:造血干细胞移植后发生神经系统症状,鉴别诊断需考虑WE的可能性,尽早给予维生素B1补充治疗以避免漏诊误诊,从而降低死亡率。
Objective： To make through introduction of Wernicke＇ s encephalopathy（ WE） following hematopoietic stem cell transplantation（ HSCT） in terms of clinical characteristics,diagnostic process and treatment.Methods： The clinical charactaristics,diagnostic and therapeutic process and prognostic follow-up in 4 patients diagnosed of WE following HSCT between January 2016 to January 2017 at Department of Hematology,Chinese Aerospace Center Hospital were retrospectively analyzed.Results： Four patients included 2 ALL and 2 AML,and 3 males and 1 female,their age ranged from 8 to 20 years old.4 patients accouted for about 3% of all petients who received HSCT at that time.Typical triad syndrome consisting of ocular motility disorders,ataxia,global confusion was seen in only 1 patient.However,confusion and heterophthongia as onset of this complication were seen in all patients.Cerebral computed tomograph scan was universally unremarkable and useless.Cerebral MRI scan disclosed that typical involvement including thalamus,fourth ventricle,third ventricle,middle cerebral aqueduct was seen in 3,while untypical site including mamillary body was in the remaining 1 patient.All received vitamin B1 supplement therapy by intramuscular injection at a dose of 100 mg each day.Initial response was observed at 2,unknown,3,4 days after treatment and all obtained complete remission within 2 weeks without any event of relapse after median follow-up period of 8（7-12） months.Conclusion： Any recipient of HSCT with clinical signs or symptoms of central nervous system should receive vitamin B1 supplementary therapy immediately to decrease risk of mortality of WE even if the diagnosis of WE is uncertain.
作者
薛松
袁磊
程昊钰
殷宇明
顾江英
张维婕
王静波
XUE SongA;YUAN Lei;CHENG Hao-Yu;YIN Yu-Ming;GU Jiang-Ying;ZHANG Wei-Jie;WANG Jing-Bo(Department of Hematology, Aerospace Center Hospital, Beijing 100049, China)
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2018年第3期880-885,共6页
Journal of Experimental Hematology
关键词
韦尼克脑病
造血干细胞移植
并发症
神经系统
Wernicke＇s encephalopathy
hematopoietic stem cell transplantation
complication
neurological system
