Home >> Exams >> AIIMS PG PD EE 2007 July Admission Notice >> ForumTopic: Lymphanagioleiomyomatosis
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| superbdoc Total Posts: 273 | Posted: Sun Oct 05, 2008 02:35 pm pls lets share info reg this hot topic.....questions are likely in AIIPPG-2009 |
| indent Total Posts: 54 | Posted: Tue Oct 07, 2008 05:30 am 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 (e.g. angiomyolipomas ) LAM cell proliferation may obstruct bronchioles, possibly leading to airflow obstruction, air trapping, formation of bullae, and pneumothoraces. Obstruction of lymphatics may result in chylothorax and chylous ascites. Obstruction of venules may result in hemosiderosis and hemoptysis. Excessive proteolytic activity, which relates to an imbalance of the elastase/alpha1-antitrypsin system or metalloprotease (MMPs) and their inhibitors (tissue inhibitors of metalloproteases [TIMPS]) may be important in lung destruction and formation of cystlike lesions. Primarily is a disease of women; rare case reports in men exist, primarily in men with tuberous sclerosis complex Primarily a disease of women of childbearing age, Range - 12 years to more than 70 years. Dyspnea, manifestations of pneumothorax, cough, chest pain, chylothorax, chyluria, pericardial effusion, pneumoperitoneu, lymphoedema may be presnet with exacerbations during menses, pregnancy, and OCP use. Facial angiofibromas, Ungual fibromas, Hypomelanotic macules, ash-leaf spot, shagreen patch, hamartomas, may be present. CA 125 may be elevated CXR - May be normal, Infiltrated, Honeycombing may be seen, pleural effusion or pneumothroax may be present Decreased diffusing capacity for carbon monoxide Spirometry - Airflow obstruction most frequent abnormality; restriction (previous pleural disease) or mixed obstruction and restriction also seen Histologic diagnosis can be made by transbronchial biopsy. HMB-45 staining is positive Medical Care Tapping of Pleural effusion, ascites, etc Bronchodilators, Oxygen, etc Hormones, Rapamycin, Doxycycline, Octreotide, etc Surgery may be necessary Lung transplantation for end stage pulmonary disease. |
| superbdoc Total Posts: 273 | Posted: Tue Oct 07, 2008 02:15 pm The explanation was worth the wait....  |