中枢神经系统朗格汉斯细胞组织细胞增生症的 15 年静脉注射免疫球蛋白治疗。
Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis.
机构信息
Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
出版信息
Acta Paediatr. 2011 Jul;100(7):e36-9. doi: 10.1111/j.1651-2227.2010.02125.x. Epub 2011 Jan 11.
AIM
There is currently no well-accepted therapy for central nervous system Langerhans cell histiocytosis (CNS-LCH), a neuroinflammatory disease clinically characterized by often progressive, neurological symptoms including ataxia, dysarthria, dysphagia, hypertonicity, intellectual impairment and behavioural abnormalities. We applied immunomodulative/anti-inflammatory treatment on a patient with progressive CNS-LCH disease.
METHOD
Intravenous immunoglobulin (IVIG) was administered monthly for 15 years to a patient with severe, image-verified neurodegenerative CNS-LCH.
RESULTS
During the IVIG treatment, the neurological deterioration initially appeared to be haltered, but over time there was still some deterioration.
CONCLUSIONS
IVIG may be beneficial in partly haltering CNS-LCH neurodegeneration, but further studies are needed.
目的
目前尚无针对中枢神经系统朗格汉斯细胞组织细胞增生症（CNS-LCH）的有效治疗方法，CNS-LCH 是一种神经炎症性疾病，临床上表现为常进行性的神经系统症状，包括共济失调、构音障碍、吞咽困难、肌肉紧张、智力障碍和行为异常。我们对一名进展性中枢神经系统 LCH 疾病患者应用免疫调节/抗炎治疗。
方法
对一名影像学证实存在严重神经退行性中枢神经系统 LCH 的患者，每月静脉注射免疫球蛋白（IVIG）治疗 15 年。
结果
在 IVIG 治疗期间，神经功能恶化最初似乎得到了控制，但随着时间的推移，仍有一定程度的恶化。
结论
IVIG 可能对部分阻止中枢神经系统 LCH 的神经退行性变有益，但仍需要进一步研究。
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