Relapses of multisystem/multifocal bone Langerhans cell histiocytosis in paediatric patients: Data analysis from the JLSG-96/02 study
Relapses of multisystem/multifocal bone Langerhans cell histiocytosis in paediatric patients: Data analysis from the JLSG-96/02 study
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DOI：
10.1111/bjh.18583
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发表时间：
2022-12-13
影响因子：
6.5
通讯作者：
Imashuku, Shinsaku
中科院分区：
文献类型：
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作者：
Sakamoto, Kenichi;Morimoto, Akira;Imashuku, Shinsaku
我们评估了复发性朗格汉斯细胞组织细胞增生症(LCH)儿童患者的复发模式，这些患者最初使用JLSG-96/02方案进行治疗。在参加JLSG-96/-02研究的317名患者中，我们分析了101名复发LCH患者的187起复发事件，其中31例为多灶骨(MFB)，70例为多系统(MS)。LCH的复发被定义为非活动期疾病(NAD)的恶化。在317例患者中，101例(31.9%)在治疗开始后1.5年首次复发。首次复发和随后复发在MFB患者和MS患者之间没有区别。在187例复发事件中，复发为单系统疾病(n=159；85%)，其中孤立性骨复发(n=104；55%)最常见。多发性硬化症复发并有器官受累的风险是极其罕见的。复发后(S)，大多数患者接受了化疗(65%)，87%的患者再次达到NAD状态。复发患者的永久性后果发生率显著高于未复发患者。在JLSG队列中，骨复发大多发生在MFB和MS患者中。大多数复发可以通过重复进行初始化疗而得到有效控制。
We assessed relapse patterns in paediatric patients with relapsed Langerhans cell histiocytosis (LCH) who were initially treated with the JLSG-96/02 protocol. We analysed 187 relapse events in 101 relapsed LCH patients [31 with multifocal bone (MFB) and 70 with multisystem (MS) at LCH diagnosis] among a total 317 patients enrolled in JLSG-96/-02 studies. Relapse of LCH was defined as an exacerbation of the non-active disease (NAD) condition. Of the 317 patients, 101 (31.9%) had the first relapse at 1.5 years after initiation of therapy. The first relapse and subsequent relapses did not differ between patients with MFB and MS disease. Of the 187 relapse events, relapse occurred as a single-system disease (n = 159; 85%), in which isolated bone relapse (n = 104; 55%) was the most common. Relapse at MS disease with the risk of organ involvement is extremely rare. After relapse(s), most patients underwent chemotherapy (122/187; 65%) and 87% of them achieved NAD status again. The incidence of permanent consequences was significantly higher in patients with relapses than in those without relapses. In the JLSG cohort, bone relapse most occurred in both MFB and MS patients. Most relapses could be effectively controlled by repeated administration of the initial chemotherapy.