International Journal of Hematology

DOI: 10.1007/s12185-018-2444-0 Pages: 192-198

Intensification of induction therapy and prolongation of maintenance therapy did not improve the outcome of pediatric Langerhans cell histiocytosis with single-system multifocal bone lesions: results of the Japan Langerhans Cell Histiocytosis Study Group-02 Protocol Study

1. Jichi Medical University, Department of Pediatrics

2. National Center for Child Health and Development, Pediatric Cancer Center

3. Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Department of Pediatrics

4. Fujita Health University School of Medicine, Department of Pediatrics

5. Aichi Gakuin University School of Pharmacy, Laboratory of Pediatrics

6. Hiroshima University Graduate School of Biomedical and Health Sciences, Department of Pediatrics

7. Kanagawa Children’s Medical Center, Division of Hematology/Oncology

8. Hyogo Prefectural Kobe Children’s Hospital, Department of Hematology and Oncology, Children’s Cancer Center

9. Juntendo University School of Medicine, Department of Pediatrics and Adolescent Medicine

10. Uji-Tokushukai Medical Center, Department of Laboratory Medicine

Correspondence to:
Akira Morimoto
Tel: +81-285-58-7366



Langerhans cell histiocytosis (LCH) with single-system (SS) multifocal bone (MFB) lesions is rarely fatal, but patients may experience relapses and develop LCH-associated sequelae. To evaluate effect on outcomes of pediatric multifocal LCH, we tested a treatment protocol modified from the Japan Langerhans Cell Histiocytosis Study Group (JLSG)-96 study. We assessed the outcomes of all consecutive newly diagnosed pediatric patients with LCH with SS-MFB lesions who were treated with JLSG-02 protocol in 2002–2009. JLSG-02 was modified from JLSG-96 as follows: increased prednisolone dosage at the induction phase and extension of maintenance therapy duration from 24 to 48 weeks. In total, 82 patients with a median follow-up duration of 8.0 years were eligible for analysis. At 6 weeks, 92.7% responded to induction; however, 27.6% of responders experienced relapses. In total, 4.8% developed central nervous system-related sequelae, including central diabetes insipidus and neurodegeneration, which were associated with relapse. None of the patients died. The 5-year event-free survival rates were not different between JLSG-02 and -96 cohort (66.7 vs. 65.1%; p = 0.697). Modification of previous treatment protocol did not contribute to improvement of outcomes in LCH with SS-MFB lesions.

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