单独肺受累朗格汉斯细胞组织细胞增生症的文献复习

Literature review of Langerhans cell histiocytosis with single lung involvement

目的总结我国2000年1月~2020年4月单独肺受累朗格汉斯细胞组织细胞增生症(LCH)患者的临床资料,以期提高临床医生对单独肺受累LCH的认识。方法通过万方、维普中文数据库和中国生物医学文献数据库,搜集2000年1月~2020年4月国内单独肺受累LCH病例,提取临床资料并进行分析。结果最终纳入文献14篇,共17例单独肺受累LCH患者,其中男15例(88.24%),女2例(11.76%)。单独肺受累LCH最常见临床表现为咳嗽(12例,70.59%),肺部影像学表现为双肺多发囊腔与结节影,中上肺野受累明显。单独肺受累LCH患者最主要的确诊方式为胸腔镜(8例,47.06%)。病理检查结果以富细胞期及增生期较为多见。免疫组化阳性结果最多见的为S-100及CD1a(各15例,88.24%)。单独肺受累LCH的误诊率高,误诊率为52.94%。治疗方面,主要采取的治疗方式为戒烟和(或)口服糖皮质激素。结论单独肺受累LCH为临床罕见疾病,男女比例为7.5∶1。咳嗽是其最常见的症状,影像学有一定特征性表现,确诊主要依据病理。对于有吸烟易患因素的单独肺受累LCH患者,应严格戒烟。靶向治疗对存在BRAF基因突变的LCH患者效果显著,对终末期患者而言,肺移植是唯一有效的手段。

Objective To summarize the clinical data of Langerhans cell histiocytosis(LCH)patients with single lung involvement from January 2000 to April 2020,in order to improve the understanding of LCH with single lung involvement by clinicians.Methods From January 2000 to April 2020,the clinical data of LCH cases with single lung involvement in China were collected by WanFang,VIP Chinese database and China Biomedical Literature Database.Results Fourteen studies were finally included,including 17 LCH patients with single lung involvement,including 15 males(88.24%)and 2 females(11.76%).The most common clinical manifestation of LCH with single lung involvement was cough(12 cases,70.59%).The pulmonary imaging findings showed multiple cysts and nodules in both lungs,with obvious involvement in the middle and upper lung fields.The most common diagnosis method for LCH patients with single lung involvement was thoracoscopy(8 cases,47.06%).The results of pathological examination were more common in the rich cell stage and the proliferative stage.The most common immunohistochemical positive results were S-100 and CD1A(15 cases each,88.24%).The misdiagnosis rate of LCH with single lung involvement was high,with a misdiagnosis rate of 52.94%.In terms of treatment,the main treatment methods were smoking cessation and/or oral glucocorticoids.Conclusion LCH with single lung involvement is a clinically rare disease,with a male/female ratio of 7.5∶1.Coughing is the most common symptom,and imaging has certain characteristics,and the diagnosis is mainly based on pathology.LCH patients with single lung involvement with smoking risk factors should be strictly smoking cessation.Targeted therapy has been shown to be effective in LCH patients with BRAF mutations,and lung transplantation is the only effective approach for end-stage patients.