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. 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. 2.0 2.1 Langerhans cell histiocytosis. Wikipedia (2015) https://en.wikipedia.org/wiki/Langerhans_cell_histiocytosis Accessed on February, 2 2016
  3. 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.
  4. 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.
  5. 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.


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