This study evaluated the outcome of patients with symptomatic bone
Langerhans cell histiocytosis (LCH) treated with
indomethacin alone, either at diagnosis or after reactivation (after recurrence with previous
therapies). We evaluated the nonrandomized use of oral
indomethacin (2 mg/kg/d) in patients with symptomatic single-system bone LCH. From 1997 to 2012, 38 sequential patients were treated for a median of 4 months. Criteria of nonactive disease (
NAD) after initial treatment (8 wk) were: no
pain, no soft tissue involvement, no increase of size, or no new bone lesions. Twenty-two patients were treated at diagnosis: 18 showed
NAD after initial treatment (2 patients who had bone reactivations were retreated with
indomethacin and remain with
NAD). Three patients improved and they are with
NAD after treatment with
indomethacin,
steroids, or
radiotherapy. One patient developed progressive
bone disease and he is with
NAD after treatment with
steroids and
chemotherapy. Sixteen patients were treated after reactivation, and all were with
NAD after initial treatment: 5 reactivated and 4 remain with
NAD after
retreatment with
indomethacin. Toxicity was not significant. We conclude that
indomethacin is a well tolerated and active
drug in patients with symptomatic
bone disease. The results support the concept that
chemotherapy may not be necessary for limited
bone disease.