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Lymphangioleiomyomatosis

Disease definition

Lymphangioleiomyomatosis (LAM) is a multiple cystic lung disease characterized by progressive cystic destruction of the lung and lymphatic abnormalities, frequently associated with renal angiomyolipomas (AMLs). LAM occurs either sporadically or as a manifestation of tuberous sclerosis complex (TSC, see this term).

ORPHA:538

  • Synonym(s):
    • LAM
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: D48.7
  • OMIM: 606690
  • UMLS: C0751674
  • MeSH: D018192
  • GARD: 3319
  • MedDRA: 10049459

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