Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan.
Department of Pediatrics, Jichi Medical University School of Medicine, Shimotsuke, Japan.
Br J Haematol. 2021 Feb;192(3):615-620. doi: 10.1111/bjh.17243. Epub 2020 Nov 25.
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.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的炎性髓系肿瘤，起源于不成熟的髓系树突状细胞，具有丝裂原活化蛋白激酶（MAPK）通路基因突变。LCH 很少致命，但患者会产生各种永久性后果（PCs）。我们报告了 JLSG-96/02 含阿糖胞苷方案治疗的儿科患者（n=317）中与 LCH 相关的 PCs 的频率。三分之一的 LCH 患者在中位随访 12 年后至少有一种 PCs。中枢神经系统（CNS）相关 PCs（神经和内分泌）占 21.5%，非 CNS 相关占 16.7%。我们需要新的治疗措施来进一步降低 LCH 相关 PCs 的频率。
