Türk Murat, Türktaş Haluk, Akyürek Nalan
Department of Chest Diseases, Gazi University Faculty of Medicine, Ankara, Turkey.
Department of Pathology, Gazi University Faculty of Medicine, Ankara, Turkey.
Turk Thorac J. 2015 Jul;16(3):154-156. doi: 10.5152/ttd.2014.4110. Epub 2014 Nov 5.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease characterized by the accumulation of histiocytes within the airspaces or parenchyma of the lung. It almost always occurs in smokers between the ages of 20 and 40. Bronchoscopic interventions, such as transbronchial biopsy (TBB) and bronchoalveolar lavage (BAL), should be performed before other more invasive procedures, but their diagnostic yield is lower. TBB is frequently non-diagnostic due to inadequate sampling. BAL cellular analysis may show alveolar macrophage predominance, and the detection of >5% of CD1a-positive cells in BAL fluid is highly suggestive and specific for the disease; however, this increase is not observed frequently. Surgical lung biopsy is the most definite modality for diagnosis. Smoking cessation must be recommended for all patients. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Here, we presented a 48-year-old smoker with an unusual and unexpected radiological presentation.
肺朗格汉斯细胞组织细胞增多症（PLCH）是一种罕见的间质性肺疾病，其特征是组织细胞在肺的气腔或实质内积聚。它几乎总是发生在20至40岁的吸烟者中。在进行其他更具侵入性的检查之前，应先进行支气管镜检查，如经支气管活检（TBB）和支气管肺泡灌洗（BAL），但其诊断阳性率较低。由于采样不足，TBB常常无法得出诊断结果。BAL细胞分析可能显示肺泡巨噬细胞占优势，BAL液中CD1a阳性细胞检测率>5%对该病具有高度提示性和特异性；然而，这种增加并不常见。外科肺活检是最确切的诊断方式。必须建议所有患者戒烟。大多数患者的预后相对较好，尤其是如果长期肺功能测试显示稳定。在此，我们介绍了一名48岁的吸烟者，其影像学表现不寻常且出乎意料。
