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Langerhans cell histiocytosis
1Department of Clinical Immunology, SGPGIMS, Lucknow.
Insights
Langerhans cell histiocytosis (LCH) is a rare childhood bone disorder. Vertebral involvement as a first sign is uncommon, as shown in three pediatric cases, including one with multifocal eosinophilic granuloma.
Area of Science:
- Pediatric oncology
- Skeletal diseases
- Histiocytosis
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder.
- It predominantly affects children, often presenting with bone pain and lesions.
Observation:
- This study presents three pediatric cases of LCH.
- Two cases were diagnosed with multifocal eosinophilic granuloma (MEG), and one with Hand-Schüller-Christian disease (HSC).
- Notably, one patient with MEG exhibited vertebral involvement as the initial symptom.
Findings:
- Vertebral involvement is an unusual presenting manifestation of LCH.
- The cases highlight the diverse skeletal manifestations of LCH in children.
- MEG can present with spinal lesions.
Implications:
- Increased awareness of spinal LCH is crucial for early diagnosis.
- Prompt recognition of vertebral LCH can prevent further complications.
- This case series contributes to understanding LCH presentations in pediatric patients.
Abstract:
Langerhans cell histiocytosis (LCH) is a rare disorder affecting predominantly children and manifesting as bone pains, bony swellings and lytic lesions. Involvement of vertebrae as presenting manifestation is unusual. Here we have presented three cases of LCH, two of multifocal eosinophilic granuloma (MEG) and one of Hand Schuller Christian disease (HSC). One of the patients with MEG; had vertebral involvement as the presenting manifestation.
