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Published on: May 1, 2015
[Lung lymphangioleiomyomatosis (LAM)]
Vesna Cukić1, Vladimir Lovre, Serif Beslić
1Klinika za plućne bolesti i TBC Podhrastovi, Sarajevo.
Medicinski Arhiv
|October 1, 2008
Summary
Lymphangioleiomyomatosis (LAM) is a rare lung disease affecting women, characterized by abnormal cell growth. This case report details a patient
Area of Science:
- Pulmonology
- Oncology
- Genetics
Background:
- Lymphangioleiomyomatosis (LAM) is an extremely rare, diffuse lung disease.
- It predominantly affects women of child-bearing age, suggesting hormonal involvement (estrogens).
- LAM involves the proliferation of smooth muscle-like cells (LAM cells) in the lungs, kidneys, and lymphatics.
Observation:
- The case involves a 43-year-old woman presenting with progressive dyspnea after pneumothorax surgery.
- Diagnosis of pulmonary LAM was confirmed via chest X-ray, CT scans, and lung biopsy.
- The patient experienced symptoms including airflow obstruction, lung cysts, and pneumothoraces.
Findings:
- LAM cell proliferation can obstruct airways and lymphatics, leading to respiratory complications.
- The disease can manifest sporadically or in association with tuberous sclerosis.
- Reported cases are rare, with approximately 400 documented worldwide.
Implications:
- Current treatment options for LAM are limited, with symptomatic therapy and hormonal treatments like progesterone being used.
- Emerging therapies, such as Rapamycin, are under investigation.
- Lung transplantation is an option for severe cases, highlighting the need for further research into effective LAM treatments.