Lymphangioleiomyomatosis (LAM) is a rare lung disease that results in a proliferation of disorderly smooth muscle growth (leiomyoma) throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). LAM occurs in a sporadic form, which only affects females, who are usually of childbearing age. LAM also occurs in patients who have tuberous sclerosis.
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Pulmonary Lymphangiomatosis is a rare but interesting condition caused by progressive widespread smooth muscle proliferation in the perilymphatic regions throughout the lungs.
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Lymphangiomyomatosis: rare lung disorder of unknown etiology mostly in women of childbearing age; proliferation of atypical smooth muscle cells in lungs, airways, blood and lymph vessels causing obstruction; symptoms include dyspnea and pneumothorax. Source: Diseases Database
Health Grades Inc.
Lymphangiomyomatosis should be considered when unexplained dyspnea, pneumothorax, pleural effusion or infiltrative radiographic changes occurs in a woman of childbearing age. Radiographic and pulmonary function peculiarities help in the diagnosis and evaluation of results of treatment. Diagnosis is dependent on lung biopsy and preferably identification of hormonal receptors. Endocrinological manipulation, in this study with medroxyprogesterone, decreased disability, morbidity and progression of this otherwise fatal illness. Early diagnosis and institution of treatment would appear to provide the best result.
National Center for Biotechnology Information, U.S. National Library of Medicine
Lymphangioleiomyomatosis (LAM) is a rare disorder resulting from proliferation in the lung, kidney, and axial lymphatics of a neoplastic cell having a smooth muscle cell phenotype (LAM cell). Cystic destruction of the lung with progressive pulmonary dysfunction and the presence of abdominal tumors (eg, angiomyolipomas [AML], lymphangioleiomyomas) characterize the disease. Because this condition typically occurs in premenopausal women, involvement of the female hormones in disease pathogenesis is a current hypothesis.
(LAM), a rare disease characterized by progressive proliferation of spindle cells, resembling immature smooth muscle, in the lung parenchyma and along lymphatic vessels in the chest and abdomen. Proliferation of spindle cells along the bronchioles leads to air trapping and the development of thin-walled cysts. Rupture of these cysts can result in pneumothorax. The spindle cell proliferation can also involve the hilar, mediastinal and extrathoracic lymph nodes, sometimes resulting in dilatation of intrapulmonary lymphatics. Involvement of the lymphatics can lead to chylous pleural effusion (see chylothorax).
Lymphangiomyomatosis occurs only in women, usually of child-bearing age. The majority present with dyspnoea. Sixty per cent develop chylous pleural effusions, and 40% develop pneumothorax. Identical clinical, radiological and pathological pulmonary changes may be seen in about 1% of patients with tuberous sclerosis. Although tuberous sclerosis affects both sexes equally, the pulmonary changes have been described almost exclusively in women. Almost all patients die within 10 years of the onset of symptoms. Recently, improved prognosis has been reported following treatment with progesterone or oophorectomy.
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Similar in pathology and x-ray appearance
Widespread proliferation of smooth muscle in pleura, alveolar septa, bronchi, pulmonary vessels and lymphatics as well as lymph nodes, especially in posterior mediastinum and retroperitoneum
Focal emphysema develops as result of narrowing of airways
Thoracic duct may be obliterated
Produce multiple small cysts with a hamartomatous proliferation of smooth muscle in their walls
A progressive disorder of women of child-bearing age, marked by nodular and diffuse interstitial proliferation of smooth muscle in the lungs, lymph nodes, and thoracic duct. Called also lymphangioleiomyomatosis.
Lymphangiomyomatosis (LAM) is a rare multi-system disorder which can occur either sporadically or in association with the tuberous sclerosis (TS) complex - approximately 1% of those with TS.
Epidemiology: It almost exclusively affects women of child bearing age 7. The estimated incidence is 1:400,000 4.
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What is the major abnormality of LAM?
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