Sandbox: Langerhans

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  • 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 multisystem Langerhans cell histiocytosis, Involves skin, lungs, bone, and GI tract Younger than 2 years of age Familial predisposition Poor prognosis