Treatment of Neurodegenerative CNS disease in Langerhans cell histiocytosis with a combination of intravenous immunoglobulin and chemotherapy
Treatment of Neurodegenerative CNS disease in Langerhans cell histiocytosis with a combination of intravenous immunoglobulin and chemotherapy
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DOI：
10.1002/pbc.21259
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发表时间：
2008-02-01
影响因子：
3.2
通讯作者：
Morimoto, Akira
中科院分区：
文献类型：
--
作者：
Imashuku, Shinsaku;Okazaki, Nagisa (Amamoto);Morimoto, Akira
背景。在极少数情况下，朗格汉斯细胞组织细胞增多症（LCH）患者会发展为神经退行性中枢神经系统疾病（ND-CNS-LCH）。ND-CNS-LCH的管理尚未建立。方法。我们用静脉注射免疫球蛋白（IVIG）和化疗（类固醇+/-长春花碱+/- 6-巯基嘌呤+/-甲氨蝶呤）联合治疗了5例儿科患者。在治疗前，5名患者中有3名患有小脑性共济失调，而其余2名有异常的MRI发现，但没有明显的神经功能障碍。IVIG每月或每月2次，剂量为250 ~ 400mg /kg/剂。结果。4名患者接受了超过23剂量的IVIG和化疗，在ND-CNS-LCH治疗开始后的30个月随访期间（中位数；范围：19+至38+），病情保持稳定，神经功能缺损或脑MRI检查未出现明显进展迹象。结论。含有ivig的治疗可能对ND-CNS-LCH有希望；然而，其有效性仍有待在更多患者和随机试验中进一步测试。中华儿科血癌杂志；2008;30(5):391 - 391。(c) 2007 Wiley-Liss, Inc。
Background. In rare cases, patients with Langerhans cell histiocytosis (LCH) develop neurodegenerative CNS disease (ND-CNS-LCH). Management of ND-CNS-LCH has not been established. Methods. We treated five pediatric patients with a combination of intravenous immunoglobulin (IVIG) and chemotherapy (steroid +/- vinblastine +/- 6-mercaptopurine +/- methotrexate). Prior to the therapy, three of the five patients had cerebellar ataxia while the remaining two had abnormal MRI findings without apparent neurological deficits. IVIG was given monthly or twice monthly at the dosage of 250-400 mg/kg/dose. Results. The four patients administered more than 23 doses of IVIG and chemotherapy remained in a stable condition and did not show significant progression signs in neurological deficits or brain MRI findings during the 30-month follow-up period (median; range: 19+ to 38+) following the initiation of therapy for ND-CNS-LCH. Conclusion. The IVIG-containing treatment may be promising for ND-CNS-LCH; however, its effectiveness remains to be further tested in more patients as well as in a randomized trial. Pediatr Blood Cancer 2008;50:308-311. (c) 2007 Wiley-Liss, Inc.