儿童造血干细胞移植后神经系统并发症的临床分析

Suxiang Liu, P. Xiao, Shaoyan Hu, H. He, Jie Li, Jun Lu, Yi Wang, Ye Lu
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引用次数: 1

摘要

目的探讨儿童造血干细胞移植(HSCT)术后神经系统(NS)并发症的发生率、临床特点及预后,进一步评价NS并发症发生的危险因素及分类特点。方法2010年10月至2018年6月,对330例HSCT患儿进行回顾性分析。结果26例患儿HSCT术后出现NS并发症。危险因素分析显示,范可尼贫血的NS并发症发生率为33.3%,骨髓增生异常/骨髓增生性肿瘤(MDS/MPN)的发生率为19.2%,急性白血病的发生率为7.3%,再生障碍性贫血的发生率为7.0%,遗传免疫缺陷病的发生率为3.1%,其他疾病的发生率均为零(P=0.027)。NS并发症患儿的中位年龄为9岁(4.75-12岁),而无NS并发症患儿的中位年龄为6岁(3-10岁)(P=0.02)。伴有和不伴有GVHD>2度的NS并发症发生率分别为24%(6/25)和6.6%(20/305)(P=0.002)。分析不同类型NS并发症,其中免疫介导性脑病53.9%,脑血管病变26.9%,移植相关血栓性微血管病(TA-TMA) 1例,药物治疗相关中毒性脑病7.7%,代谢性脑病3.8%,周围神经病变7.7%。NS并发症死亡率为34.6%,其中免疫介导性脑病死亡率最高(13/19,68.4%)。然而,与药物治疗相关的中毒性脑病、代谢性脑病和周围神经病变患者在HSCT后均存活。无NS并发症的患儿总生存率高于有NS并发症的患儿。结论HSCT术后NS并发症的发生率与原发疾病、年龄、GVHD >2度相关。与药物治疗相关的中毒性脑病、代谢性脑病和周围神经病变患儿预后优于免疫介导性脑病和TA-TMA患儿。减少神经系统并发症可能提高HSCT后儿童的长期生存率。关键词:儿童;造血干细胞移植;神经系统并发症;风险因素;长期生存
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Clinical analysis of nervous system complications after hematopoietic stem cell transplantation in children
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. Key words: Children; Hematopoietic stem cell transplantation; Nervous system complications; Risk factors; Long term survival
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