颌骨朗格汉斯细胞组织细胞增多症：68例临床分析

Langerhans cell histiocytosis of the jaw: clinical analysis of 68 cases.

作者信息

Li Jiale, Wu Hao, Wang Zilin, Han Jing, Liu Jiannan, Han Bing

机构信息

Department of Oral and Maxillofacial Surgery, SchoolandHospitalofStomatology, Jilin University, No. 1500, Qing Hua Road, Changchun, 130021, China.

Department of Oral and Maxillofacial Head and Neck Oncology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, No. 639, Zhi Zao Ju Road, Shanghai, 200011, China.

出版信息

Orphanet J Rare Dis. 2025 Apr 21;20(1):191. doi: 10.1186/s13023-025-03680-w.

DOI:10.1186/s13023-025-03680-w

PMID:40259405

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12013012/

Abstract

BACKGROUND

This study aims to investigate the clinical characteristics, imaging features, treatment, and prognostic factors of jaw Langerhans cell histiocytosis (JLCH), providing valuable insights for its clinical diagnosis and management.

METHOD

This study retrospectively analyzed the clinical and follow-up data of JLCH patients treated between January 2010 and January 2024. Data collected included gender, age, symptoms, imaging findings, treatment strategies, and outcomes. Univariate and multivariate Cox regression analyses were performed using SAS software to identify factors affecting treatment outcomes, with P ≤ 0.05 considered statistically significant.

RESULTS

A total of 68 patients (50 males, 18 females; median age 13.5 years) were included. Forty percent of patients were under 10 years old, and 71% had mandibular involvement. Disease classification included 49 cases of single-system unifocal (SS-s) disease, 10 cases of single-system multifocal (SS-m) disease, and 9 cases of multi-system (MS) disease. Common symptoms included jaw or tooth pain (28 cases), facial swelling (22), gingival ulceration (10), and loose teeth (9). Imaging revealed periodontal disease-like (7), cyst-like (17), and osteomyelitis-like (44) lesions. Univariate and multivariate Cox regression analyses identified that female patients had a lower risk of progression (P = 0.014, HR 0.071), while SS-m (P = 0.019, HR 4.992) and MS patients (P = 0.030, HR 4.182) exhibited higher progression risks compared to SS-s patients. Cyst-like (P = 0.001, HR 0.054) and osteomyelitis-like lesions (P < 0.001, HR 0.023) were associated with lower progression risks compared to alveolar lesions.

CONCLUSION

JLCH can affect individuals of all ages, though it is more common in children. Factors such as gender, lesion multiplicity, and lesion type (alveolar) are significant in predicting disease progression. Complete surgical resection combined with radiotherapy offers the highest likelihood of cure for SS-type JLCH.

摘要

背景

本研究旨在探讨颌骨朗格汉斯细胞组织细胞增多症（JLCH）的临床特征、影像学特征、治疗方法及预后因素，为其临床诊断和管理提供有价值的见解。

方法

本研究回顾性分析了2010年1月至2024年1月期间接受治疗的JLCH患者的临床和随访数据。收集的数据包括性别、年龄、症状、影像学表现、治疗策略和结果。使用SAS软件进行单因素和多因素Cox回归分析，以确定影响治疗结果的因素，P≤0.05被认为具有统计学意义。

结果

共纳入68例患者（男性50例，女性18例；中位年龄13.5岁）。40%的患者年龄在10岁以下，71%的患者下颌骨受累。疾病分类包括49例单系统单病灶（SS-s）疾病、10例单系统多病灶（SS-m）疾病和9例多系统（MS）疾病。常见症状包括颌骨或牙痛（28例）、面部肿胀（22例）、牙龈溃疡（10例）和牙齿松动（9例）。影像学检查发现牙周病样病变（7例）、囊肿样病变（17例）和骨髓炎样病变（44例）。单因素和多因素Cox回归分析确定，女性患者进展风险较低（P = 0.014，HR 0.071），而与SS-s患者相比，SS-m患者（P = 0.019，HR 4.992）和MS患者（P = 0.030，HR 4.182）进展风险较高。与牙槽病变相比，囊肿样病变（P = 0.001，HR 0.054）和骨髓炎样病变（P < 0.001，HR 0.023）进展风险较低。