Long‐term complications in uniformly treated paediatric Langerhans histiocytosis patients disclosed by 12 years of follow‐up of the JLSG‐96/02 studies
Long‐term complications in uniformly treated paediatric Langerhans histiocytosis patients disclosed by 12 years of follow‐up of the JLSG‐96/02 studies
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JLSG-96/02 研究的 12 年随访揭示了接受统一治疗的儿童朗格汉斯组织细胞增多症患者的长期并发症
DOI：
10.1111/bjh.17243
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发表时间：
2020
影响因子：
6.5
通讯作者：
S. Imashuku
中科院分区：
文献类型：
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作者：
K. Sakamoto;A. Morimoto;Y. Shioda;T. Imamura;S. Imashuku
郎格汉斯细胞组织细胞增生症(LCH)是一种罕见的炎症性髓系肿瘤，起源于带有丝裂原活化蛋白激酶(MAPK)途径基因突变的未成熟髓系树突状细胞。LCH很少是致命的，但患者会出现各种永久性后果(PC)。我们报告了接受JLSG-96/02AraC方案治疗的儿童患者(n=317)LCH相关PC的频率。三分之一的LCH患者在平均12年的随访期内至少有一台PC。与中枢神经系统(CNS)相关的PC(神经和内分泌)占21.5%，非CNS相关的PC占16.7%。我们需要新的治疗方法来进一步降低LCH相关PC的频率。
Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia derived from immature myeloid dendritic cells with the mitogen‐activated protein kinase (MAPK) pathway gene mutation. LCH is rarely fatal, but patients develop various permanent consequences (PCs). We report the frequencies of LCH‐related PCs in paediatric patients (n = 317) treated by the JLSG‐96/02 AraC‐containing regimens. One‐third of LCH patients had at least one PC at a median follow‐up of 12 years. Central nervous system (CNS)‐related PCs (neurological and endocrinological) accounted for 21·5%, non‐CNS‐related 16·7%. We require novel therapeutic measures to further reduce the frequency of LCH‐related PCs.
DOI：
--
发表时间：
2007
期刊：
影响因子：
--
作者：
Morimoto A;Mizutani S;et. al.
通讯作者：
et. al.