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Predictors of outcome in children with Langerhans cell histiocytosis
Rima F Jubran1, Araz Marachelian, Frederick Dorey
1Keck School of Medicine, University of Southern California, Division of Pediatric Hematology/Oncology, Children's Hospital Los Angeles, USA. rjubran@chla.usc.edu
Background:
Our goal was to examine the clinical course of patients with Langerhans cell histiocytosis (LCH), with a special emphasis on bone disease and to attempt to identify and examine the factors that may predict reactivation and overall prognosis.
Procedure:
We conducted a retrospective chart review of 132 consecutive pediatric patients treated at Children's Hospital Los Angeles for LCH from 1984 to 2001.
Results:
The risk for reactivation after initial management is significantly higher for patients with multiple bone and those with multiple organ involvement as compared with patients who had a single bone lesion (hazard ratios are 7.1 and 11.6). Patients younger than 1 year in the multiple organ group have an increased risk of death at 2 years when compared with the older patients in that group (hazard ration = 6.2, P = 0.022). Endocrine abnormalities were seen in 20% and 7.5% of patients with or without skull lesions respectively.
Conclusions:
Patients with LCH involving only the bones have a significantly better outcome than those with other organ involvement. Patients with multiple organ involvement who are less than 1 year of age are at high risk of death and should be approached more aggressively upfront.