Langerhans cell histiocytosis
Langerhans cell histiocytosis, abbreviated LCH, is a rare genetic disorder of tissue macrophages. It broadly fits into the category of histiocytoses. It used to known as eosinophilic granuloma. It has been referred to by several eponyms - Hand-Schüller-Christian disease, Abt-Letterer-Siwe disease, and histiocytosis X.
General
- Looks like pulmonary Langerhans cell histiocytosis - see medical lung diseases.
Microscopic
Features:
- Langerhans cells histiocytes - key feature.
- Clusters of cells (histiocytes) with a reniform (kidney-shaped) nucleus and abundant foamy cytoplasm.
- Nucleus may look like a "coffee bean", i.e. have nuclear grooves (similar to those in papillary thyroid carcinoma) -- appearance dependent on the rotation of the nucleus.[1]
- Chromatin pattern: fine granular, light gray.
- Clusters of cells (histiocytes) with a reniform (kidney-shaped) nucleus and abundant foamy cytoplasm.
- +/-Eosinophils - often prominent.
Images:
- WC:
- www:
DDx:
- Kimura disease - eosinophilia.
- See lymph node pathology.
IHC
Electron microscopy
Etiology:
- Cell membrane invagination.[3]
Appearance:
- Electron dense, cytoplasmic tennis racket-like body.
Images:
See also
References
- ↑ BN. 15 March 2011.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 604862
- ↑ URL: http://path.upmc.edu/cases/case147/micro.html. Accessed on: 7 January 2012.
- ↑ URL: http://path.upmc.edu/cases/case298.html. Accessed on: 14 January 2012.