Relapses of multisystem/multifocal bone Langerhans cell histiocytosis in paediatric patients: Data analysis from the JLSG-96/02 study |
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Authors: | Kenichi Sakamoto Akira Morimoto Yoko Shioda Toshihiko Imamura Shinsaku Imashuku the Japan LCH Study Group |
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Institution: | 1. Department of Pediatrics, Shiga University of Medical Science, Otsu, Japan;2. Department of Pediatrics, Jichi Medical University School of Medicine, Shimotsuke, Japan;3. Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan;4. Department of Pediatrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan;5. Division of Laboratory Medicine, Uji-Tokushukai Medical Center, Uji, Japan |
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Abstract: | 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. |
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Keywords: | bone JLSG Langerhans cell histiocytosis relapse |
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