34例首发为耳颞部症状朗格罕氏细胞组织细胞增生症临床分析  

An Analysis of 34 Cases of Langerhans Cell Histiocytosis with Initial Otological Symptoms

作　　者：李越[1] 叶放蕾[1] LI Yue;YE Fanglei(Department of Otology,First Affiliated Hospital of Zhengzhou University,Zhengzhou 450000,China)

机构地区：[1]郑州大学第一附属医院耳科,郑州450000

出　　处：《中华耳科学杂志》2021年第4期582-586,共5页Chinese Journal of Otology

摘　　要：目的探讨首发为耳颞部症状的朗格罕氏细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床特点。方法回顾性分析郑州大学第一附属医院2011年11月-2019年8月期间收治并经病理确诊的34例以耳颞部症状为首发的LCH患者的临床资料,总结其性别、发病年龄、临床表现、影像学检查、诊断、治疗及预后等临床特点。结果本研究患者多为儿童,男女比例1.4:1,多为单系统受累,最常见耳颞部症状为耳漏和耳颞部肿物。大于2岁患者组(A组)、小于等于2岁患者组(B组)两组耳颞部症状差异无统计学意义(P>0.05),MS-LCH组和SS-LCH组耳颞部症状差异无统计学意义(P>0.05)。多累及乳突和外耳道。预后较好,A、B两组疗效之间差异无统计学意义(P>0.05),SS-LCH组疗效优于MS-LCH组(P<0.05)。结论首发为耳颞部症状的LCH最常累及乳突和外耳道,主要表现为耳颞部肿物和耳漏,耳颞部症状的表现形式与年龄和临床分类无关,SS-LCH患者预后优于MS LCH。患者年龄≤2岁不是影响预后的危险因素。Objective To report clinical characteristics of Langerhans cell histiocytosis(LCH)presented with initial otologic symptoms.Methods Clinical data of 34 patients with LCH confirmed by pathology and initial otologic symptoms admitted to the First Affiliated Hospital of Zhengzhou University from November 2011 to August 2019 were analyzed retrospectively for clinical characteristics including gender,age of disease onset,clinical manifestations,imaging data,diagnosis,treatment and outcomes.Results Most patients in this study were children with a male to female ratio of 1.4:1.Single system involvement was most common,and the most common symptoms were otorrhea and ear/temporal bone mass with no significant difference between children older than 2 years and those under 2 years(P>0.05)or between patients with multiple system involvement(MS-LCH)and single system involvement(SS-LCH)(P>0.05).Mastoid and external auditory meatus were most commonly involved.Treatment outcomes were generally good with no significant difference between children older than 2 years and those under 2 years(P>0.05),although the rate of cure was better for SS-LCH was than for MS-LCH(P<0.05).Conclusion LCH with initial otologic symptoms most often involves the mastoid and external auditory canal with are ear/temporal bone mass and otorrhea as main manifestations,which are not related to age or clinical classification.Prognosis is better for SS-LCH than for MS-LCH and young age(≤2 years)is not a risk factor for worse prognosis.

关 键 词：朗格罕氏细胞组织细胞增生症 耳颞部 预后 

分 类 号：R764[医药卫生—耳鼻咽喉科]