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Langerhans cell histiocytosis with orbital involvement
T R Kramer1, R J Noecker, J M Miller
1Department of Ophthalmology, University of Arizona Health Sciences Center, Tucson 85719, USA. TKramer@eyes.Arizona.edu
American Journal of Ophthalmology
|December 24, 1997
Summary
Three children in one community were diagnosed with Langerhans cell histiocytosis (LCH), a rare disease, showing a significantly increased incidence. This suggests LCH may signal environmental exposures.
Area of Science:
- Pediatric Oncology
- Epidemiology
- Ophthalmology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of indeterminate dendritic cells.
- Orbital involvement in LCH, while uncommon, can lead to significant morbidity in children.
Observation:
- Three pediatric cases of LCH with orbital and intracranial involvement presented within a short timeframe in a single geographic community.
- Clinical presentation included rapidly progressive unilateral proptosis in 2-year-old children.
- Radiographic findings revealed enhancing lesions involving the orbit, sphenoid bone, and temporal lobe.
Findings:
- The observed incidence rate in this cluster was approximately 26 times the expected rate, suggesting a localized environmental trigger.
- Treatment with vincristine and prednisone yielded variable responses.
Implications:
- The high incidence and spatial-temporal clustering of LCH cases suggest LCH may serve as a sentinel disease for identifying unusual environmental exposures.
- Further epidemiological and environmental investigations are warranted to explore potential causative factors.