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Adult pulmonary Langerhans cell histiocytosis

Disease definition

Adult pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare histiocytic lung disease characterized by the accumulation of Langerhans and other inflammatory cells in the small airways, resulting in the formation of nodular inflammatory lesions.

ORPHA:99874

  • Synonym(s):
    • Pulmonary histiocytosis X
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: C96.5
  • OMIM: -
  • UMLS: C1455705
  • MeSH: -
  • GARD: -
  • MedDRA: -

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