Nomura S, Minami S, Fujimura M, Yasui M, Nakao S, Hayashi S
Department of Internal Medicine, Inami General Hospital, 938 Inami, Inami-machi, Higashitonami-gun, Toyama, 932-0211 Japan.
Nihon Kokyuki Gakkai Zasshi. 2001 Dec;39(12):944-8.
A 52-year-old woman complained of exertional dyspnea. She had two sons with tuberous sclerosis and presented with facial angiofibroma and bilateral renal angiomyolipomas. Her chest radiograph demonstrated diffuse reticular shadows and chest CT revealed numerous well-defined cysts outlined by thin walls. Pulmonary lymphangiomyomatosis was diagnosed by transbronchial biopsy that showed multifocal proliferation of immature smooth muscle cells located in the walls of some bronchi, and in alveolar walls and pulmonary vessels. Immunoreactivity for SMA and HMB 45 was present in the same cells, but immunostaining for estrogen and progesterone receptors was negative. Much is still unknown about pulmonary lymphangiomyomatosis and pulmonary involvement in tuberous sclerosis. Further studies, such as gene analysis, will clarify the relationship between these diseases.
一名52岁女性主诉劳力性呼吸困难。她有两个患有结节性硬化症的儿子，本人有面部血管纤维瘤和双侧肾血管平滑肌脂肪瘤。她的胸部X线片显示弥漫性网状阴影，胸部CT显示有许多边界清晰、薄壁勾勒的囊肿。经支气管活检诊断为肺淋巴管平滑肌瘤病，活检显示一些支气管壁、肺泡壁和肺血管壁中有未成熟平滑肌细胞的多灶性增殖。同一细胞中存在平滑肌肌动蛋白（SMA）和HMB 45免疫反应性，但雌激素和孕激素受体免疫染色为阴性。关于肺淋巴管平滑肌瘤病以及结节性硬化症的肺部受累情况，仍有许多未知之处。进一步的研究，如基因分析，将阐明这些疾病之间的关系。
