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[Child Langerhans cell histiocytosis]
Jean Donadieu1, Sébastien Héritier1
1Hôpital Trousseau, centre de référence des histiocytoses, service d'hémato-oncologie pédiatrique, 26, avenue du Dr-Netter, 75012 Paris, France.
Presse Medicale (Paris, France : 1983)
|January 15, 2017
Summary
Langerhans cell histiocytosis (LCH) is a rare disease affecting multiple organs, with current treatments offering a 98% survival rate in children. However, long-term complications like diabetes insipidus can occur in up to 30% of cases.
Area of Science:
- Pediatric Oncology
- Histiocytosis
- Rare Diseases
Background:
- Langerhans cell histiocytosis (LCH) is a rare multisystemic disorder characterized by pathological tissue biopsy and clinical/radiological involvement.
- LCH commonly affects bone, skin, and the pituitary gland, but can impact any organ system, including the liver, spleen, lungs, and central nervous system.
- The disease's natural history is highly variable, ranging from self-resolving lesions to severe, multi-organ involvement with potentially fatal outcomes and permanent sequelae.
Purpose of the Study:
- To summarize the key aspects of Langerhans cell histiocytosis (LCH), including its definition, common manifestations, and natural history.
- To emphasize the importance of a multidisciplinary approach in managing LCH due to its systemic nature and variable presentation.
- To highlight current therapeutic outcomes and the prevalence of long-term complications in pediatric LCH cases.
Main Methods:
- Review of current understanding of LCH definition, epidemiology, and natural history.
- Analysis of treatment outcomes based on current protocols.
- Assessment of the incidence of long-term sequelae in treated pediatric patients.
Main Results:
- Current treatment protocols achieve a 98% survival rate in pediatric LCH cases.
- Despite high survival rates, up to 30% of children experience long-term sequelae.
- Common sequelae include diabetes insipidus, hormonal deficits, and deafness; less common but severe complications include respiratory failure and sclerosing cholangitis.
Conclusions:
- Langerhans cell histiocytosis (LCH) requires a coordinated, multidisciplinary approach for optimal management.
- While survival rates are high, vigilance for and management of long-term complications are crucial in pediatric LCH patients.
- Effective treatment protocols significantly improve outcomes, but ongoing monitoring for sequelae is essential.
