Rawlins Frederic A, Hull James E, Morgan Julia A, Morris Michael J
Internal Medicine Residency, Department of Medicine (MCHE-MDX), San Antonio Military Medical Center, 3551 Roger Brooke Drive, Fort Sam Houston, TX 78234-6200, USA.
Case Rep Med. 2013;2013:637232. doi: 10.1155/2013/637232. Epub 2013 Aug 27.
Langerhans cell histiocytosis (LCH) is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed.
朗格汉斯细胞组织细胞增多症(LCH)是一种主要影响年轻成年吸烟者的罕见疾病。其特征是朗格汉斯细胞异常增殖,朗格汉斯细胞是一种特殊的单核细胞 - 巨噬细胞谱系抗原呈递细胞。LCH可单独累及肺部,也可作为全身性疾病的一部分。最常见的是,该病在第三或第四个十年出现,无性别差异。症状通常包括呼吸困难和咳嗽。通常,体格检查无明显异常,但在疾病晚期可能观察到肺心病。胸部X线片通常异常,表现为结节状或间质性浸润以及囊性改变。成年吸烟者中上叶出现这些表现的胸部高分辨率计算机断层扫描实际上可诊断LCH。肺功能评估结果不一。很少有哮喘与这种疾病相关的报道。仅有三例关于LCH诊断时同时伴有哮喘诊断的报道。我们报告一例患者,该患者出现哮喘的体征和症状,包括气道高反应性的确诊结果。在患者对哮喘的常规治疗无反应并完成进一步评估后,确诊为LCH。