There remain differing opinions among experts as to whether it is definitively a cancer or not
About
- Very rare disease
- Typically affects children and young adults
- Proliferative disorder of histiocytes
- Paediatric form - Eosinophilic granuloma
Aetiology
- Mutations of the BRAF gene in about half the cases
- Produce too many Langerhans cells or histiocytes
- There is granuloma formation in multiple organs
- Affects Skin and nails, mouth, bones
- Lymph nodes, pituitary gland, and thyroid gland
Variants
- Hashimoto-Pritzker disease, a congenital self-healing form
- Letterer-Siwe disease, a severe, acute and disseminated form
- Hand-Schüller-Christian disease, an intermediate chronic form with multiple lesions characterized by diabetes insipidus, bulging of the eye and localized lesions in the bone
- Eosinophilic granuloma, a less severe form, is characterized by solitary or few, and chronic lesions of bone or other organs.
Clinical
- Lytic Skull lesions
- Pneumothorax, Lung cyst formation, Haemoptysis
- Cerebellar demyelination
- Hypothalamic dysfunction
- Diabetes Insipidus
Investigations
- Skull X-Ray and CT shows Lytic skull lesions
- Biopsy shows eosinophils and granulomas formation with Langerhan's cells
- involvement of granulomas in the apices leads to cyst formation and pneumothorax and haemoptysis
- Hypernatraemia - DI
Differential
Management
- Steroids are indicated for symptomatic disease
- Histiocytosis X is smoking-related and smoking must stop
- Progression leads to death from respiratory failure in 10%
- Treatment for LCH varies and may include surgery, chaemotherapy, radiation therapy, and the use of certain medications.
References