Lymphangioleiomyomatosis

Lymphangioleiomyomatosis

Updated: 11/01/2021

© Jun Wang, MD, PhD

 

General features

  • Cystic destruction with progressive dysfunction of lung
  • Proliferation of lymphatic smooth muscle-like cells
  • Probably originated from perivascular epithelioid cell
  • Most common in reproductive age women
  • Associated with tuberous sclerosis

Key clinical features

  • Most common presentations: Dyspnea, cough, pleural effusion, etc
  • Pneumothorax commonly seen

Key morphological features

Genetic abnormalities

  • TSC1 and TSC2

Diagnosis

  • Radiologic studies: Cystic changes
  • Pathological identification of LAM cells

Markers

  • Positive: HMB45

Treatment

  • Treatments of complications
  • Lung function improvement
  • Lung transplantation

 

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Contents

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Anemia

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Lymphoid neoplasms

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