Quraishi M S, Blayney A W, Walker D, Breatnach F B, Bradley P J
ENT Department, University Hospital, Nottingham, United Kingdom.
Head Neck. 1995 May-Jun;17(3):226-31. doi: 10.1002/hed.2880170310.
Langerhans' cell histiocytosis (LCH) is an uncommon, poorly understood granulomatous disease, characterized by the idiopathic proliferation of Langerhan's cells or their marrow precursors. In 1985, the Philadelphia Work-shop adopted the term "Langerhans' cell histiocytosis" (LCH) to differentiate it from reactive and neoplastic causes of histiocytosis.
This study includes 73 pediatric patients diagnosed with this condition in Dublin, Ireland, and Nottingham, England, during a 34-year period (1959 to 1993). These patients are reviewed with respect to clinical presentation, difficulty with making a histological diagnosis, their management, and outcome.
A total of 49 patients (67%) had head and neck involvement. Bony involvement was the most frequent sign, most frequently located in the skull. There were 11 deaths (15%) in this series, all associated with multisystem disease, and nine of these deaths were in children younger than 2 years of age.
The role of otolaryngologists is important in the early and accurate evaluation, staging, and diagnosis of LCH. It may mimic more common diseases, such as otitis externa, acute mastoiditis, skin rash, gingivitis, or cervical lymphadenopathy. Patients with multisystem disease may be so ill at presentation that the head and neck lesions may be overlooked. The current management of LCH has become increasingly conservative, and in the 1990s, fewer cases are given chemotherapy or radiotherapy. The prognosis is very good for single-system disease and poor for multisystem disseminated disease with early onset.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见且了解甚少的肉芽肿性疾病，其特征为朗格汉斯细胞或其骨髓前体细胞的特发性增殖。1985年，费城研讨会采用了“朗格汉斯细胞组织细胞增多症”（LCH）这一术语，以将其与组织细胞增多症的反应性和肿瘤性病因相区分。
本研究纳入了在34年期间（1959年至1993年）在爱尔兰都柏林和英国诺丁汉被诊断为此病的7名儿科患者。对这些患者的临床表现、组织学诊断的困难程度、治疗及预后进行了回顾。
共有49例患者（67%）有头颈部受累。骨受累是最常见的体征，最常位于颅骨。本系列中有11例死亡（15%），均与多系统疾病相关，其中9例死亡发生在2岁以下儿童。
耳鼻喉科医生在LCH的早期准确评估、分期和诊断中发挥着重要作用。它可能会模仿更常见的疾病，如外耳道炎、急性乳突炎、皮疹、牙龈炎或颈部淋巴结病。多系统疾病患者在就诊时可能病情很重，以至于头颈部病变可能被忽视。目前对LCH的治疗越来越保守，在20世纪90年代，接受化疗或放疗的病例减少。单系统疾病的预后非常好，而早发性多系统播散性疾病的预后很差。
