Sandbox: Langerhans
- 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 | Smoking | Good prognosis with smoking cessation |
Unifocal Langerhans cell histiocytosis | Isolated bone involvement | Children | 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 unisystem Langerhans cell histiocytosis, | Involves skin, lungs, bone, and GI tract | Younger than 2 years of age | Familial predisposition | Poor prognosis |