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