Predictors of outcome in children with Langerhans cell histiocytosis

• Published in: Pediatric Blood & Cancer Vol. 45; no. 1; pp. 37 - 42
• Main Authors: Jubran, Rima F., Marachelian, Araz, Dorey, Frederick, Malogolowkin, Marcio
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.07.2005; Wiley
• Subjects: Adolescent; Adult; Biological and medical sciences; Bone Diseases - diagnosis; Bone Diseases - mortality; Child; Child, Preschool; children; Disease Progression; Disease-Free Survival; Female; General aspects; histiocytosis; Histiocytosis, Langerhans-Cell - complications; Histiocytosis, Langerhans-Cell - diagnosis; Histiocytosis, Langerhans-Cell - mortality; Humans; Infant; Los Angeles - epidemiology; Male; Medical sciences; outcome; Prognosis; Recurrence; Retrospective Studies; Survival Rate; Tumors; Los Angeles; Human; Pediatrics; Cancerology; Prognosis; Langerhans cell histiocytosis; children; Hemopathy; Predictive factor; Child; histiocytosis; outcome
• ISSN: 1545-5009; 1545-5017; 1096-911X
• DOI: 10.1002/pbc.20364

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. Pediatr Blood Cancer 2005; 45:37–42. © 2005 Wiley‐Liss, Inc.