Langerhans cell histiocytosis
Langerhans cell histiocytosis
Updated: 08/30/2018
© Jun Wang, MD, PhD
General features
- Idiopathic
- Likely neoplastic
Clinical presentations
- Tumor like lesions
- Nodal or extranodal
Key risk factors
- Smoking for pulmonary lesions
Key pathogenesis
- Activating BRAF mutation
Key morphological features
- Partial effacement of lymph node
- Solid infiltration of Langerhan cells distending sinus
- Langerhan cells: Elongated nuclei with nucleargrooves/folds
- Birbeck granules: Electron microscopic finding, elongated zipperlike cytoplasmic structure, double outer sheath
Genetic abnormalities
- Activating BRAF mutation
Negative prognosis indicator
- Wide spread type: Letterer-Siwe disease
- Extreme age
- Lung involvement
Back to contents
Comments
Post a Comment