Multiinstitutional Trials in the Treatment of Pediatric Patients with Langerhans Cell Histiocytosis. Therapeutic Results Treated by Protocol JLSG-96.:Therapeutic Results Treated by Protocol JLSG-96
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We report here the therapeutic results of LCH patients with multifocal lesions treated by the protocol JLSG-96. Seventy-eight newly diagnosed and 12 pretreated/relapsed patients were analyzed. They were registered from June 1996 to February 2001, with median follow-up of 22.8 months (range 4.7-58 months). The newly diagnosed LCH patients (33 with SM-type and 45 with MM-type) were treated by protocol A, a standard protocol for induction and maintenance therapy. In SM-type LCH, CR was obtained in 32 of the 33 patients (97%), reactivation was noted in 30%, and the probabilities of a 4-year survival by Kaplan-Meier analysis were relapse-free 68%, overall 100%. Diabetes insipidus (DI) was noted in only one case (3%). In MM-type LCH, CR, the reactivation and probabilities of a 4-year relapse-free survival were 51, 22, and 34%, respectively. The overall survival was 97.5%, with one death. DI was noted in 6 cases (13%). Forty relapsed or refractory LCH patients (including the 12 pretreated ones, 17 nonresponders and 11 relapsed among the newly diagnosed, 9 with SM-type, and 31 with MM-type) were treated by protocol B, engineered as a salvage therapy. CR was obtained in 100% of SM patients and in 42% of MM patients. In summary, the CR rates of newly diagnosed multifocal LCH patients treated by JLSG-96 (both protocol A and B) were 100 and 74%, respectively. These therapeutic results, particularly induction rates, were comparable to those of the European large-scale LCH studies reported previously. But the relapse rate of SM-type LCH was high, and the entire mortality rate (2%) was significantly better for patients treated with our protocol.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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