The authors all state that there is no potential conflicts of interest.
Nationwide survey of single-system single site Langerhans cell histiocytosis in Japan†
Article first published online: 2 SEP 2009
Copyright © 2009 Wiley-Liss, Inc.
Pediatric Blood & Cancer
Volume 54, Issue 1, pages 98–102, January 2010
How to Cite
Morimoto, A., Ishida, Y., Suzuki, N., Ohga, S., Shioda, Y., Okimoto, Y., Kudo, K. and Ishii, E. (2010), Nationwide survey of single-system single site Langerhans cell histiocytosis in Japan. Pediatr. Blood Cancer, 54: 98–102. doi: 10.1002/pbc.22224
- Issue published online: 9 NOV 2009
- Article first published online: 2 SEP 2009
- Manuscript Accepted: 2 JUL 2009
- Manuscript Received: 19 JAN 2009
- Ministry of Health, Labor and Welfare, Japan
- Langerhans' cell histiocytosis;
- single system
Since neither a standard treatment nor a protocol study for single-system single site (SS-s)-type Langerhans cell histiocytosis (LCH) exists, we conducted a nationwide survey in Japan to clarify the epidemiology and clinical outcome of this subtype.
Questionnaires regarding the clinical course of children with SS-s-type LCH diagnosed between 1995 and 2006 were sent to all members of the Japanese Society of Pediatric Hematology.
One hundred forty-six children with histologically proven SS-s LCH were evaluable. The most frequently affected organ was bone (82%), followed by skin (12%). Few patients (14%) had a CNS-RISK lesion defined by the Histiocyte Society. Patients with a skin lesion were diagnosed at a significantly younger age than patients with a bone lesion (median: 6 months vs. 5 years 11 months, P < 0.001). The treatment regimen varied, but one-third of the patients in total and 71% of patients with a CNS-RISK lesion received chemotherapy that did not include etoposide. All but one patient attained remission. Ten patients (7%) showed reactivation. Of these, all eight with an initial bone lesion only exhibited reactivation in the bone(s). One patient with an initial skin lesion exhibited reactivation in the thymus. None of the patients died from disease progression or treatment complications.
Our retrospective study, in which a relatively large proportion of the patients received chemotherapy, reveals that patients with SS-s LCH have a good prognosis. A prospective study should be conducted to confirm this and to identify the most effective and least toxic therapy for SS-s LCH. Pediatr Blood Cancer 2010; 54:98–102. © 2009 Wiley-Liss, Inc.