Diffuse reticular interstitial infiltrations accompanied by hyperinflation. |
Kye Young Lee, Young Whan Kim, Sung Koo Han, Young Soo Shim, Keun Youl Kim, Yong Chol Han |
Department of lnternal Medicine, Seoul National University College of Medicine, Seoul, Korea |
과팽창이 동반된 미만성 망상형 간질성 폐침윤 |
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Abstract |
A 38-year-old female was admitted due to progressive exertional dyspnea and intermittent blood-tinged sputum. Chest PA showed diffuse reticular infiltration accompanied by hyperinflation. Pa0 2 was normal in resting state but profoundly decreased during exercise. Spirometry showed severe obstructive pattern but DLCO was markedly decreased. Lung volumes measured by helium equilib. rium method was increased. On HRCT, numerous and relatively uniform sized cysts were evenly distributed throughout the bilateral lung fields. Open lung biopsy was performed and pulmonary lyæphangioleiomyomatosis (LAM) was diagnosed based on the findings of abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and small pulmonary veins. Immunohistochemical staining was negative for estrogen receptor but positive for progesterone receptor. Medroxyprogesterone therapy was initiated. |
Key Words:
Lymphangioleiomyomatosis, reticular, hyperinflation |
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