TY - JOUR
T1 - Outcome of pediatric patients with Langerhans cell histiocytosis treated with 2 chlorodeoxyadenosine
T2 - A nationwide survey in Japan
AU - Imamura, Toshihiko
AU - Sato, Takashi
AU - Shiota, Yoko
AU - Kanegane, Hirokazu
AU - Kudo, Kazuko
AU - Nakagawa, Shinichirou
AU - Nakadate, Hisaya
AU - Tauchi, Hisamichi
AU - Kamizono, Junji
AU - Morimoto, Akira
N1 - Funding Information:
Acknowledgments The authors thank all of the physicians who participated in this study. This work was supported by a Grant for Clinical Cancer Research and Research on Measures for Intractable Diseases from the Japanese Ministry of Health, Labour and Welfare.
PY - 2010/5
Y1 - 2010/5
N2 - The aim of this study was to assess the outcome of treatment with 2-chlorodeoxyadenosine (2-CdA) in pediatric patients with Langerhans cell histiocytosis (LCH) in Japan. We retrospectively identified 17 pediatric LCH patients treated with 2-CdA. All patients were refractory or reactivated cases who had been initially treated according to the JLSG-02 protocol of the Japan LCH study group. At initiation of 2-CdA therapy, 4 patients had primary refractory multisystem (MS) disease with risk organ (RO) involvement (MS?), 9 patients had reactivated MS disease [5 MS+ and 4 without RO involvement (MS-)], and the remaining 4 patients had refractory/reactivated multifocal bone disease (MFB). Treatment with 2-CdA(4-9 mg/m2/day) was administered on 2-5 consecutive days and repeated every 3-4 weeks for a period that ranged from 2 to 12 months. Four primary refractory patients were treated with 2-CdA combined with high dose of cytarabine. In MS+ patients, response to treatment was observed in 5 of the 9 patients. In MS-/MFB patients, 5 of the 8 patients showed response to treatment. In the patients who were primary refractory or had reactivation during initial chemotherapy, 4 of 10 patients showed good response. On the other hand, in the patients having reactivation while off therapy, 6 of 7 patients showed good response. These findings suggest that 2-CdA is effective for reactivated LCH while off therapy.
AB - The aim of this study was to assess the outcome of treatment with 2-chlorodeoxyadenosine (2-CdA) in pediatric patients with Langerhans cell histiocytosis (LCH) in Japan. We retrospectively identified 17 pediatric LCH patients treated with 2-CdA. All patients were refractory or reactivated cases who had been initially treated according to the JLSG-02 protocol of the Japan LCH study group. At initiation of 2-CdA therapy, 4 patients had primary refractory multisystem (MS) disease with risk organ (RO) involvement (MS?), 9 patients had reactivated MS disease [5 MS+ and 4 without RO involvement (MS-)], and the remaining 4 patients had refractory/reactivated multifocal bone disease (MFB). Treatment with 2-CdA(4-9 mg/m2/day) was administered on 2-5 consecutive days and repeated every 3-4 weeks for a period that ranged from 2 to 12 months. Four primary refractory patients were treated with 2-CdA combined with high dose of cytarabine. In MS+ patients, response to treatment was observed in 5 of the 9 patients. In MS-/MFB patients, 5 of the 8 patients showed response to treatment. In the patients who were primary refractory or had reactivation during initial chemotherapy, 4 of 10 patients showed good response. On the other hand, in the patients having reactivation while off therapy, 6 of 7 patients showed good response. These findings suggest that 2-CdA is effective for reactivated LCH while off therapy.
KW - 2-Chlorodeoxyadenosine
KW - Langerhans cell histiocytosis
KW - Reactivation
UR - http://www.scopus.com/inward/record.url?scp=77954541268&partnerID=8YFLogxK
U2 - 10.1007/s12185-010-0558-0
DO - 10.1007/s12185-010-0558-0
M3 - Article
C2 - 20361277
AN - SCOPUS:77954541268
SN - 0925-5710
VL - 91
SP - 646
EP - 651
JO - International Journal of Hematology
JF - International Journal of Hematology
IS - 4
ER -