儿童造血干细胞移植后神经系统并发症的临床分析  

Clinical analysis of nervous system complications after hematopoietic stem cell transplantation in children

作　　者：刘素香[1] 肖佩芳[1] 胡绍燕[1] 何海龙[1] 李捷[1] 卢俊[1] 王易[1] 陆叶[1] 胡昳歆 Liu Suxiang;Xiao Peifang;Hu Shaoyan;He Hailong;Li Jie;Lu Jun;Wang Yi;Lu Ye;Hu Yixin(Department of Hematology&Oncology,Children's Hospital,Soochow University,Suzhou 215000,China)

机构地区：[1]苏州大学附属儿童医院血液肿瘤科,苏州215000

出　　处：《中华器官移植杂志》2019年第11期691-695,共5页Chinese Journal of Organ Transplantation

基　　金：苏州市临床重点专病(LCZX201507);苏州市民生科技项目(SYSD201756,SYS201643);苏州市儿童白血病重点实验室(SZS201615)。

摘　　要：目的分析儿童造血干细胞移植(HSCT)后神经系统(NS)并发症的发病情况、临床特点及预后,评估HSCT后儿童受者发生NS并发症的危险因素及类型特点。方法回顾性分析2010年10月至2018年6月330例接受HSCT儿童受者的临床资料。结果330例受者HSCT后共26例发生NS并发症。危险因素分析显示,范可尼贫血、骨髓增生异常综合征/骨髓增殖性肿瘤(MDS/MPN)、急性白血病、再生障碍性贫血、先天性免疫缺陷病、其他疾病发生NS并发症的发生率分别为33.3%(2/6)、19.2%(5/26)、7.3%(14/191)、7.0%(4/57)、3.1%(1/32)、0(0/18);发生与未发生NS并发症受者的中位年龄分别为9岁(4.75~12岁)和6岁(3~10岁)(P=0.02);伴和不伴有2度以上移植物抗宿主病(GVHD)受者发生NS并发症的发生率分别为24%(6/25)和6.6%(20/305)(P=0.002)。NS并发症类型临床分析显示,免疫相关性脑病占53.9%(14/26),脑血管病变占26.9%(7/26),其中1例为移植相关性血栓性微血管病(TA-TMA),药物相关中毒性脑病占7.7%(2/26),代谢性脑病占3.8%(1/26),外周神经病变占7.7%(2/26)。26例受者的病死率为34.6%(9/26),其中发生免疫相关性脑病的病死率最高,为68.4%(13/19),发生药物相关中毒性脑病、代谢性脑病、外周神经病变的患儿均存活。生存分析显示,HSCT后未合并NS并发症患儿的总体存活率高于合并NS并发症的患儿。结论HSCT后NS并发症的发生率与原发疾病、年龄、2度以上GVHD有关。NS并发症中以药物相关中毒性脑病、代谢性脑病、外周神经病变预后佳,并发免疫相关性脑病、TA-TMA病死率高。减少NS并发症可能有助提高儿童受者的长期存活率。Objective To explore the incidence,clinical characteristics and prognosis of nervous system(NS)complications after hematopoietic stem cell transplantation(HSCT)in children and further evaluate the occurring risk factors of NS complications and category characteristics.Methods From October 2010 to June 2018,retrospective analysis was performed for 330 HSCT children.Results Twenty-six children developed NS complications after HSCT.Risk factor analysis revealed that the incidence of NS complications were 33.3%in Fanconi anemia,19.2%in myelodysplastic/myeloproliferative neoplasm(MDS/MPN),7.3%in acute leukemia,7.0%in aplastic anemia,3.1%in genetic immunodeficiency disease,none in other disease(P=0.027).The median age of children with NS complications was 9(4.75-12)versus 6(3-10)(P=0.02)those without NS complications.The incidence of NS complications with and without GVHD>2 degree were 24%(6/25)and 6.6%(20/305)(P=0.002).Different types of NS complications were analyzed,including 53.9%immune-mediated encephalopathy,26.9%cerebrovascular lesion,one case of transplantation associated thrombotic microangiopathy(TA-TMA),7.7%drug therapy-related toxic encephacopathy,3.8%metabolic encephalopathy and 7.7%peripheral neuropathy.The mortality of NS complications was 34.6%and those with immune-mediated encephalopathy had the highest mortality(13/19,68.4%).However,those with drug therapy-related toxic encephacopathy,metabolic encephalopathy and peripheral neuropathy all survived after HSCT.The overall survival rate was higher in children without NS complications than those with NS complications.Conclusions The incidence of NS complications after HSCT is correlated with primary diseases,age and GVHD>2 degree.Children with drug therapy-related toxic encephacopathy,metabolic encephalopathy and peripheral neuropathy have better prognosis than those with immune-mediated encephalopathy and TA-TMA.Reducing NS complications may improve long-term survival of children after HSCT.

关 键 词：儿童 造血干细胞移植 神经系统并发症 危险因素 长期存活 

分 类 号：R7[医药卫生—临床医学] R4