Steffelaar J W, Nijkamp D A, Hilvering C
Scand J Respir Dis. 1977 Apr;58(2):103-9.
A case is reported of pulmonary lymphangiomyomatosis in 44-year-old woman presenting with progressive exertional dyspnoea, unproductive cough and haemoptysis. The patient showed no symptoms or signs of tuberous sclerosis, a condition often found in association with pulmonary lymphangiomyomatosis. A pulmonary biopsy specimen showed disruption of alveolar septa and fibrosis. Subpleural lymph vessels and pulmonary venules showed cuffs of spindle-shaped cells. The leiomyomatous nature of these cells was evident from their appearance and staining reactions with light microscopy, and the presence of smooth muscle antigens as demonstrated by immunofluorescence technique. Since the lesion is not restricted to lymphatics, but can also be present in blood vessels, the condition may more appropriately be designated as pulmonary angiomyomatosis.
报告了一例44岁女性的肺淋巴管平滑肌瘤病，该患者表现为进行性劳力性呼吸困难、干咳和咯血。患者未表现出结节性硬化症的症状或体征，而结节性硬化症常与肺淋巴管平滑肌瘤病相关。肺活检标本显示肺泡间隔破坏和纤维化。胸膜下淋巴管和肺小静脉可见梭形细胞套。这些细胞的平滑肌瘤性质从其外观和光镜下的染色反应以及免疫荧光技术显示的平滑肌抗原的存在可明显看出。由于病变不仅局限于淋巴管，也可存在于血管中，因此该病症可能更恰当地称为肺血管平滑肌瘤病。
