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 organs involvement into 4 groups: pulmonary Langerhans cell histiocytosis, unifocal Langerhans cell histiocytosis, multifocal unisystem Langerhans cell histiocytosis, and multifocal multisystem Langerhans cell histiocytosis.
The table below lists the features of each specific Langerhans cell histiocytosis subtype:


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