Morimoto Akira, Ikushima Satoshi, Kinugawa Naoko, Ishii Eiichi, Kohdera Urara, Sako Masahiro, Fujimoto Junichiro, Bessho Fumio, Horibe Keizo, Tsunematsu Yukiko, Imashuku Shinsaku
Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan.
Cancer. 2006 Aug 1;107(3):613-9. doi: 10.1002/cncr.21985.
The treatment outcome of multifocal childhood Langerhans cell histiocytosis (LCH) has not been satisfactory and has resulted in poor therapeutic responses with high mortality and a high incidence of reactivation with late sequelae. To overcome these issues, the Japan LCH Study Group-96 (JLSG-96) protocol was conducted prospectively from 1996 to 2001 in Japan.
Newly diagnosed children with multifocal LCH were classified into 2 groups: a single-system multisite (SS-m) group and a multisystem (MS) group. All patients initially were treated on Protocol A, which consisted of 6 weeks of induction therapy with combined cytosine arabinoside, vincristine (VCR), and prednisolone (PSL) followed by 6 months of maintenance therapy. Patients who had a poor response to the induction of Protocol A were switched to a salvage regimen (Protocol B), which consisted of an intensive combination of doxorubicin, cyclophosphamide, VCR, and PSL.
In total, 91 patients were treated, including 32 patients in the SS-m group and 59 patients in the MS group. At the median 5-year follow-up, 96.9% of patients in the SS-m group and 78.0% of patients in the MS group had good response status. Diabetes insipidus developed in 3.1% of patients in the SS-m group and in 8.9% of patients in the MS group. The overall survival rate at 5 years for the SS-m and MS groups was 100% and 94.4% +/- 3.2%, respectively.
The JLSG-96 protocol attained very low mortality for pediatric patients with multifocal LCH.
儿童多灶性朗格汉斯细胞组织细胞增多症（LCH）的治疗效果一直不尽人意，导致治疗反应不佳、死亡率高以及复发率高并伴有晚期后遗症。为克服这些问题，日本LCH研究组-96（JLSG-96）方案于1996年至2001年在日本进行了前瞻性研究。
新诊断的多灶性LCH患儿分为两组：单系统多部位（SS-m）组和多系统（MS）组。所有患者最初接受方案A治疗，该方案包括6周的诱导治疗，联合使用阿糖胞苷、长春新碱（VCR）和泼尼松龙（PSL），随后进行6个月的维持治疗。对方案A诱导治疗反应不佳的患者改用挽救方案（方案B），该方案由阿霉素、环磷酰胺、VCR和PSL的强化联合组成。
总共治疗了91例患者，其中SS-m组32例，MS组59例。在中位5年随访时，SS-m组96.9%的患者和MS组78.0%的患者反应良好。SS-m组3.1%的患者和MS组8.9%的患者发生了尿崩症。SS-m组和MS组5年总生存率分别为100%和94.4%±3.2%。
JLSG-96方案使多灶性LCH儿科患者的死亡率非常低。
