累及喉部的进行性结节性组织细胞增生症1例并文献复习

Laryngeal involvement in progressive nodular histiocytosis:a case report with literature review

目的探讨1例罕见的累及喉部的进行性结节性组织细胞增生症的诊疗过程、临床及病理特点。方法提取患者的病例资料、病理切片,对切片进行免疫组化染色观察,总结本病历的特点并查询PNH相关文献。结果本病历中患者的病理提示梭形细胞弥漫浸润,免疫组化显示CD68和CD163阳性。患者出现声门上狭窄、气管壁增厚、气管内多发结节,曾因呼吸困难行气管切开,累及皮肤形成多发结节,面部结节形成"狮面"外观,确诊后予以安罗替尼药物治疗可减缓病情发展。结论该病通过病理检查确诊,可累及喉部引起呼吸困难,面部结节除非严重影响功能,不主张手术切除皮损。

Objective To explore the diagnosis and treatment,the clinical characteristic,as well as the pathological features,of a rare case of progressive nodular histiocytosis involving the larynx.Methods Patient case data,pathological sections were extracted and observed by immunohistochemical staining to summarize the characteristics of this case and to consult the literature related to PNH.Results The pathology of the patient in this case suggested a diffuse infiltration of spindle cells,and immunohistochemistry was positive for CD68 and CD163.The patient presented with supraglottic stenosis,thickening of the tracheal wall,and multiple nodules in the trachea.The patient had undergone a tracheotomy due to dyspnea.This disease involves the skin,and it manifests in multiple nodules and facial nodules that give rise to a“lion face”appearance.After diagnosis,treatment with anlotinib can delay the development of this disease.Conclusion The disease is diagnosed based on pathological examination.It can involve the larynx and cause breathing difficulties.Surgical removal of the facial nodules is not recommended unless body function is seriously affected.