Langerhans cell histiocytosis classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Langerhans cell histiocytosis may be classified according to the extent of organ involvement into 4 groups: pulmonary Langerhans cell histiocytosis, unifocal Langerhans cell histiocytosis, multifocal unisystem Langerhans cell histiocytosis, and multifocal multisystem Langerhans cell histiocytosis.[1][2]
Classification
- Langerhans cell histiocytosis may be classified according to the extent of organ involvement into 4 groups: pulmonary Langerhans cell histiocytosis, unifocal Langerhans cell histiocytosis, multifocal unisystem Langerhans cell histiocytosis, and multifocal multisystem Langerhans cell histiocytosis.[1][2]
- The table below lists the features of each specific Langerhans cell histiocytosis subtype:[3][4][5]
| Subtype | Organ Involvement | Age | Risk Factors | Prognosis |
|---|---|---|---|---|
| Pulmonary Langerhans cell histiocytosis | Isolated pulmonary involvement | Adults | Occurs exclusively among smokers | Good prognosis with smoking cessation |
| Unifocal Langerhans cell histiocytosis | Isolated bone involvement | 2–10 years of age | Familial predisposition | Good prognosis, may spontaneously regress |
| Multifocal unisystem Langerhans cell histiocytosis | Triad of diabetes insipidus, exopthalmos, and lytic bone lesions | 2–10 years of age | Familial predisposition | Good prognosis, may spontaneously regress |
| Multifocal multisystem Langerhans cell histiocytosis | Involves skin, lungs, bone, and GI tract | < 2 years of age | Familial predisposition | Poor prognosis |
References
- ↑ 1.0 1.1 Langerhans cell histiocytosis. Libre Pathology (2015) http://librepathology.org/wiki/index.php/Langerhans_cell_histiocytosis#cite_note-10 Accessed on February, 3 2016
- ↑ 2.0 2.1 Langerhans cell histiocytosis. Wikipedia (2015) https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis Accessed on February, 2 2016
- ↑ Elia D, Torre O, Cassandro R, Caminati A, Harari S (June 2015). "Pulmonary Langerhans cell histiocytosis: a comprehensive analysis of 40 patients and literature review". Eur. J. Intern. Med. 26 (5): 351–6. doi:10.1016/j.ejim.2015.04.001. PMID 25899682.
- ↑ Goldsmith AJ, Myssiorek D, Valderrama E, Patel M (January 1993). "Unifocal Langerhans' cell histiocytosis (eosinophilic granuloma) of the petrous apex". Arch. Otolaryngol. Head Neck Surg. 119 (1): 113–6. PMID 8417735.
- ↑ Imashuku S, Kinugawa N, Matsuzaki A, Kitoh T, Ohki K, Shioda Y, Tsunematsu Y, Imamura T, Morimoto A (November 2009). "Langerhans cell histiocytosis with multifocal bone lesions: comparative clinical features between single and multi-systems". Int. J. Hematol. 90 (4): 506–512. doi:10.1007/s12185-009-0420-4. PMID 19779766.