Jeelani Hafiz Muhammad, Ehsan Hamid, Sheikh Muhammad Mubbashir, Riaz Adeel, Mahboob Hafiz
Internal Medicine, Rosalind Franklin University of Medicine and Science, McHenry, USA.
Internal Medicine, MedStar Union Memorial Hospital, Baltimore, USA.
Cureus. 2020 Sep 11;12(9):e10377. doi: 10.7759/cureus.10377.
Langerhans cell histiocytosis (LCH), formally referred to histiocytosis X, is a histiocytic disorder with unknown etiology. The pathogenesis is believed to originate from myeloid dendritic cells and is now considered an inflammatory myeloid neoplasm within the revised 2016 Histiocyte Society classification. Pulmonary Langerhans cell histiocytosis (PLCH) is a rare and isolated form of LCH with a strong affiliation with smoking in adults of 20-40 years of age. Characteristic CT chest and histologic findings are instrumental in the early recognition and management of a disease. We herein report a case of a Caucasian smoker female with a significant history of interstitial lung disease (ILD) presented with recurrent and progressive worsening dyspnea. History of ILD and recurring respiratory symptoms raised suspicion of PLCH. CT chest and pathological findings confirmed the diagnosis, and discontinuation of smoking resulted in favorable clinical outcomes.
朗格汉斯细胞组织细胞增多症（LCH），以前称为组织细胞增多症X，是一种病因不明的组织细胞疾病。其发病机制被认为起源于髓样树突状细胞，在2016年修订的组织细胞协会分类中，现在被认为是一种炎症性髓样肿瘤。肺朗格汉斯细胞组织细胞增多症（PLCH）是LCH的一种罕见且孤立的形式，与20至40岁成年人的吸烟密切相关。胸部CT特征和组织学表现有助于疾病的早期识别和管理。我们在此报告一例有显著间质性肺疾病（ILD）病史的白种人吸烟女性，她出现反复且进行性加重的呼吸困难。ILD病史和反复出现的呼吸道症状引发了对PLCH的怀疑。胸部CT和病理结果证实了诊断，戒烟带来了良好的临床结果。
