Yokoyama A, Kohno N, Sakai K, Kondo K, Hirasawa Y, Hiwada K
Second Department of Internal Medicine, Ehime University School of Medicine.
Intern Med. 1997 Jun;36(6):413-6. doi: 10.2169/internalmedicine.36.413.
Cystic lesion, malignancy and interstitial pneumonia are well-known as pulmonary complications of patients with von Recklinghausen's disease. We report herein an unusual patient with distal acinar emphysema and interstitial pneumonia of prominent hypercellularity demonstrated by transbronchial biopsy and broncho-alveolar lavage fluid (BALF). Six months after quitting smoking, the total cell count of BALF was remarkably reduced. This patient remains stable under 5-year observation in terms of symptoms and findings of both BALF and pulmonary function tests. Quitting smoking may have facilitated a favorable prognosis for the particular lung disease complicated in this patient.
囊肿性病变、恶性肿瘤和间质性肺炎是冯·雷克林霍增氏病患者众所周知的肺部并发症。我们在此报告一例不寻常的患者，其经支气管活检和支气管肺泡灌洗术（BALF）显示有远端腺泡型肺气肿和具有显著细胞增多的间质性肺炎。戒烟六个月后，BALF的总细胞计数显著降低。在对该患者进行的5年观察中，其症状、BALF检查结果和肺功能测试结果均保持稳定。戒烟可能有助于该患者所并发的特定肺部疾病获得良好预后。
