Department of Pathology, 1University of Health Sciences Turkey, Haseki Training and Research Hospital, ISTANBUL, TURKEY.
Turk Patoloji Derg. 2023;39(3):179-184. doi: 10.5146/tjpath.2022.01591.
Pulmonary Langerhans cell histiocytosis is a cystic lung disease characterized by the proliferation of parenchymal dendritic cells. The disease can become chronic or even cause pulmonary fibrosis. Our aim in this study was to investigate the typical histological findings and interstitial fibrosis in pulmonary Langerhans cell histiocytosis cases.
In the study, cases that had undergone diagnostic resection were screened. Smoking, histological stage (subacute, subacute-chronic), and cystic and eosinophilic granulomas were confirmed in the cases. In addition to emphysema, chronic nonspecific bronchiolitis, interstitial fibrosis (subpleural-paraseptal fibrosis, peribronchial fibrosis, fibrotic nonspecific interstitial pneumonia), honeycomb-type fibrocysts, and unexpected lesions were investigated. Descriptive and comparative (Fisher exact test) statistical analyses were used in the study (p < 0.05).
A total of 27 cases were detected; age distribution was 17-68 (36.4). Smoking was present in 15 (55.5%) cases. Six (22.2%) cases were subacute, and 21 (7.7%) cases were subacute-chronic histological stage. A cystic lesion was present in 22 (81.4%) cases. All cases had emphysema accompanying the underlying lesions. Chronic nonspecific bronchiolitis was detected in 14 (51.8%) cases. Interstitial fibrosis was detected in 8 (29.6%) patients. Compared to interstitial fibrosis and nonfibrosis, there was no significant difference between being younger than 39 years, gender, smoking, and histological stage (p=0.41; 1; 0.69; 0.63, respectively).
There is a risk of developing interstitial fibrosis patterns and honeycomb-type fibrocysts in the progression of pulmonary Langerhans cell histiocytosis. Histopathological evaluation can play an important role in the detection of risk groups.
肺朗格汉斯细胞组织细胞增生症是一种以实质树突状细胞增生为特征的囊性肺疾病。该疾病可发展为慢性，甚至导致肺纤维化。本研究旨在探讨肺朗格汉斯细胞组织细胞增生症病例的典型组织学表现和间质纤维化。
对接受诊断性切除的病例进行筛选。在病例中确认吸烟、组织学分期（亚急性、亚急性-慢性）、囊性和嗜酸性肉芽肿。除肺气肿、慢性非特异性细支气管炎、间质纤维化（胸膜下-间隔旁纤维化、支气管周围纤维化、纤维化非特异性间质性肺炎）、蜂窝状纤维囊、意外病变外，还进行了研究。研究采用描述性和比较（Fisher 确切检验）统计分析（p<0.05）。
共发现 27 例病例；年龄分布为 17-68 岁（36.4）。15 例（55.5%）有吸烟史。6 例（22.2%）为亚急性，21 例（7.7%）为亚急性-慢性组织学分期。22 例（81.4%）存在囊性病变。所有病例均伴有基础病变的肺气肿。14 例（51.8%）发现慢性非特异性细支气管炎。8 例（29.6%）发现间质纤维化。与无间质纤维化相比，年龄小于 39 岁、性别、吸烟和组织学分期之间无显著差异（p=0.41；1；0.69；0.63）。
在肺朗格汉斯细胞组织细胞增生症的进展中，存在间质纤维化模式和蜂窝状纤维囊的风险。组织病理学评估在检测高危人群中可能发挥重要作用。
