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[Pulmonary lymphangiomyomatosis].

A Meleniewska-Maciszewska1, P Krakówka, S Radowicki

  • 1I Kliniki Gruźlicy i Chorób Płuc Instytutu Gruźlicy i Chorób Płuc w Warszawie.

Pneumonologia I Alergologia Polska
|January 1, 1992
PubMed
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Pulmonary lymphangiomyomatosis (PLAM) in a menopausal woman presented diagnostic challenges. Despite varied hormonal therapies and treatments for pleural effusion, the disease progressed over eight years.

Area of Science:

  • Pulmonology
  • Oncology
  • Endocrinology

Background:

  • Pulmonary lymphangiomyomatosis (PLAM) is a rare, progressive disease characterized by the proliferation of smooth muscle cells in the lungs.
  • It primarily affects women of childbearing age, and presentation during menopause is uncommon.

Observation:

  • A menopausal woman with PLAM experienced diagnostic difficulties with lung biopsies.
  • The diagnosis was confirmed by the identification of lymph seepage into the pleural cavity.
  • The patient developed bilateral pleural effusions, treated with drainage, cytotoxic therapy, and hormonal interventions.

Findings:

  • The patient underwent treatment with prednisolone, tamoxifen, methylprogesterone, and danazol.
  • Despite multimodal therapy, high-resolution CT revealed extensive pulmonary changes indicating disease progression over 8 years.

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  • The patient's functional status remained relatively preserved, without requiring oxygen therapy.

    • Implications:

    • This case highlights the diagnostic challenges of PLAM, particularly in post-menopausal women.
    • It underscores the complex, long-term management strategies required for advanced PLAM.
    • Further research into hormonal therapies and disease progression in atypical presentations of PLAM is warranted.