Lymphangioleiomyomatosis (LAM). A Review of Clinical and Morphological Features.:A Review of Clinical and Morphological Features
スポンサーリンク
概要
- 論文の詳細を見る
A review is presented of the clinical and morphological manifestations of lymphangioleiomyomatosis (LAM), a systemic disorder of unknown etiology that affects women. The clinical features include dyspnea, hemoptysis, recurrent pneumothorax, chylothorax, and chylous ascites. It is characterized by: 1) proliferation of abnormal smooth muscle cells (LAM cells) in pulmonary interstitium and along the axial lymphatics of the thorax and abdomen; 2) thin-walled pulmonary cysts, and 3) a high incidence of angiomyolipomas. The pulmonary cystic lesions have a characteristic appearance on high resolution computed tomography. The most specific method for diagnosing LAM is lung biopsy to demonstrate the presence of LAM cells, either by their characteristic histological appearance or by specific immunostaining with HMB-45 antibody. LAM cells differ in several important respects from the types of smooth muscle cells normally present in lung. Their reactivity with HMB-45 antibody is localized in stage I and stage II melanosomes. LAM cells show additional evidence of incomplete melanogenesis, and the significance of these observations remains to be determined. Two types of LAM cells are recognized: 1) small, spindle-shaped cells that are centrally located in the LAM nodules and are highly immunoreactive for matrix metalloproteinase-2 (MMP-2), its activating enzyme (MT-1-MMP), and proliferating cell nuclear antigen (PCNA), and 2) large, epithelioid cells that are distributed along the periphery of the nodules and show a high degree of immunoreactivity with HMB-45 antibody and with antibodies against estrogen and progesterone receptors. Types of treatment used for LAM include oophorectomy, administration of Lupron or progesterone and in very severe cases, pulmonary transplantation (following the onset of respiratory insufficiency, not relieved by O<SUB>2</SUB>).
- 日本医科大学医学会の論文
日本医科大学医学会 | 論文
- わが国における腸チフスの疫学的考察--1973年〜1976年届出菌検出例を中心として
- 臨床および実験報告 二絨毛膜双胎妊娠における高年初産の検討
- 救急災害医学の確立と展望[含 略歴 研究業績] (定年退職教授記念講演会講演要旨)
- 臨床のために 関節リウマチの診断と治療
- TNFファミリーの制御による関節リウマチ治療の可能性 (特集 第15回公開「シンポジウム」(アレルギー・膠原病に対する新たな展開))