Report two case of lymphangioleiomyomatosis (LAM) with presentation of recurrent pneumothorax

Manouchehr Aghajanzadeh 1, *, Ali Alavi Foumani 2, Azita Tangestaninejad 2, Ali Asghar Fakhrmousavi 2, Zahra Sadin 2, Peram Taleby 2, Omid Mosaffaee -Rad 1, Ehsan Hajipour 3 and Mohaya Farzin 3

1 Department of Thoracic Surgery, Guilan University of Medical Sciences, Rasht, Iran.
2 Department of Internal Medicine, Inflammatory Lung Diseases Research Center, Razi Hospital, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran.
3 Department of physiology, Razi Clinical Research Development Center, Guilan University of Medical Sciences, Rasht, Iran.
 
Research Article
International Journal of Scholarly Research in Biology and Pharmacy, 2023, 03(02), 009–016​
Article DOI: 10.56781/ijsrbp.2023.3.2.0039
Publication history: 
Received on 29 September 2023; revised on 27 November 2023; accepted on 30 November 2023
 
Abstract: 
Pulmonary Lymphangioleiomyomatosis (LAM) is a rare and female-dominant disease and associated with smooth muscle cell proliferation, (LAM) which presents with diffuse progressive destruction of the pulmonary parenchyma. The incidence is less than 1 per million, which results in cystic lung disease and presente commonly with dyspnea and pneumothorax. Work up for diagnosis and exclusion other disease as: Sjögren’s syndrome type A, B and mazement of angiotensin-converting enzyme (ACE) levels, alpha-1-antitrypsin levels, and vascular endothelial growth factor (VEGF) anti bodies. Definitive diagnosis limited only to tissue sampling. No effective treatments are currently proposed for this disease. Two 49 and 39year-old woman who was referred with recurrent pneumothorax, after thoracotomy and resection, diagnosed was made with pulmonary Lymphangioleiomyomatosis. After pulmonary Lymphangioleiomyomatosis was performed, they treated with anti-estrogen therapy and symptomatic supportive care. In follow up pneumothorax was not occurred again. Pulmonary Lymphangioleiomyomatosis is a very rare disease that cannot be effectively cured.
 
Keywords: 
Pulmonary Lymphangioleiomyomatosis; Pathological examination; Immunohistochemistry; Anti estrogen therapy​
 
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