Department of Pediatric Oncology, Cambridge University Hospital NHS Trust, Cambridge, United Kingdom.
Pediatr Blood Cancer. 2013 Oct;60(10):E118-21. doi: 10.1002/pbc.24591. Epub 2013 May 29.
In an international study of systemic childhood ALCL, 12/463 patients had CNS involvement, three of which had isolated CNS disease. Comparative analysis of CNS positive and negative patients showed no difference in ALK positivity, immunophenotype, presence of B symptoms or other sites of disease. The lymphohistiocytic variant was over represented in the CNS positive group (36% vs. 5%). With multi-agent chemotherapy, including high dose methotrexate, Ara-C and intrathecal treatment, the event free and overall survival of the CNS positive group at 5 years were 50% (95%CI, 25-75%) and 74% (45-91%), respectively with a median follow up of 4.1 years.
在一项国际系统性儿童 ALCL 研究中，463 例患者中有 12 例出现中枢神经系统受累，其中 3 例为孤立性中枢神经系统疾病。对中枢神经系统阳性和阴性患者的比较分析显示，ALK 阳性率、免疫表型、B 症状存在或其他部位疾病无差异。淋巴组织细胞变异型在中枢神经系统阳性组中占比过高（36% vs. 5%）。采用多药物化疗，包括大剂量甲氨蝶呤、阿糖胞苷和鞘内治疗，中枢神经系统阳性组的 5 年无事件生存率和总生存率分别为 50%（95%CI，25-75%）和 74%（45-91%），中位随访时间为 4.1 年。
