Predictors of outcome in children with Langerhans cell histiocytosis. Pediatr Blood Cancer 2005 Jul;45(1):37-42
Date
03/16/2005Pubmed ID
15768381DOI
10.1002/pbc.20364Scopus ID
2-s2.0-20344383983 (requires institutional sign-in at Scopus site) 91 CitationsAbstract
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.
Author List
Jubran RF, Marachelian A, Dorey F, Malogolowkin MMESH terms used to index this publication - Major topics in bold
AdolescentAdult
Bone Diseases
Child
Child, Preschool
Disease Progression
Disease-Free Survival
Female
Histiocytosis, Langerhans-Cell
Humans
Infant
Los Angeles
Male
Prognosis
Recurrence
Retrospective Studies
Survival Rate
