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Paediatric pulmonary Langerhans cell histiocytosis
Mhairi Barclay1, Rebecca Devaney1, Jayesh M Bhatt2
1Paediatric Respiratory Medicine, Nottingham University Hospitals NHS Trust, Nottingham, UK.
Breathe (Sheffield, England)
|July 21, 2020
Summary
Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare lung disease in children, often part of multisystem LCH. Diagnosis requires histology, and management focuses on chemotherapy and respiratory complications.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Histiocytosis
Background:
- Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease.
- Unlike adult LCH, pPLCH is rarely isolated and often a component of multisystem LCH.
Purpose of the Study:
- To provide an overview of paediatric pulmonary LCH.
- To discuss the differential diagnosis of paediatric cystic lung disease.
- To focus on diagnosis, monitoring, and management of pPLCH complications.
Main Methods:
- Review of current literature on paediatric pulmonary LCH.
- Emphasis on histological and immunophenotypic diagnostic criteria.
- Discussion of clinical and radiological features.
Main Results:
- pPLCH diagnosis relies on integrated clinical, radiological, and pathological findings.
- LCH is now considered an inflammatory myeloid neoplasia.
- Pulmonary involvement does not worsen LCH prognosis.
Conclusions:
- pPLCH requires a multidisciplinary approach for optimal outcomes.
- Complications like pneumothoraces and respiratory failure are life-threatening.
- Prognosis is favorable but impacted by multisystem disease.
