Langerhan Cell Histocytosis: Clinical Features, Oral Manifestation, Diagnosis, and Treatment

藍格罕細胞組織球增生症臨床及口腔病徵，診斷及治療

黃則達(Tze-Ta Huang) ； 劉景勳(Chiang-Shin Liu) ； 王東堯(Tung-Yiu Wong) ； 陳畊仲(Ken-Chung Chen) ； 施皓(Hao Shih) ； 陳建旭(Jiann-Shiuh Chen) ； 黃振勳(Jehn-Shyun Huang)

《臺灣口腔顎面外科學會雜誌》 28卷1期 (2017/03) Pp. 34-47

摘要

Langerhans cell histiocytosis (LCH) is a rare disease. The pathogenesis of LCH is under ongoing investigations to determine whether LCH is a reactive or neoplastic process. The LCH is characterized by intense and abnormal proliferation of Langerhans cells. The patients might present with the whole body systemic involvement, or only local manifestations. The LCH can be clinically divided into three groups: unifocal, multifocal unisystem, and multifocal multisystem. Our patient was a 28-year-old male, presented with severe dry cough, progressive dyspnea on exertion, poor appetite and bodyweight loss at first. Pulmonary Langerhans cell histiocytosis was diagnosed via video-assisted thoracoscopic surgery biopsy. After eight months, oral LCH lesions were confirmed based on clinical, radiographic, and pathological examination. The disease progressed to multifocal multisystem LCH. The patient further received chemotherapy at our Department of Pediatric Oncology. The systemic condition was improved after treatment, however, oral LCH exacerbatedly posed localized periodontal destruction and local adjuvant radiotherapy was suggested. But the patient lost follow-up and did not receive subsequent treatment. The prognosis of LCH is excellent for single-focus disease; by contrast, with multi-focal disease more than half have a chronic course with poor result. Herein, we reported a case of LCH with its clinical manifestation, diagnosis, and treatment.

關鍵字

Langerhans cell histiocytosis ； Oral ； Diagnosis ； Treatment

並列摘要

藍格罕細胞組織球增生症是一個罕見的系統性疾病，其致病機轉仍待進一步研究去區分出爲反應增生或癌化病變。藍格罕細胞組織球異常增生爲其病灶特徵，病患可能出現全身多系統或單一局部病灶。藍格罕細胞組織球增生症可依臨床症狀區分爲單一病灶，單一系統多病灶，和多系統多病灶三種。本篇報告爲一位二十八歲男性，最初因運動時喘，沒有胃口，體重減輕而至高雄榮總求診，肺部切片顯示爲肺部藍格罕細胞組織球增生症，病症發生八個月後出現口腔病症，進而疾病進展爲多系統多病灶。病人後於成大醫院小兒血液腫瘤科求診，進一步診斷後並接受化學治療，其症狀獲得控制。後因多處大臼齒齒槽骨喪失動搖，於口腔顎面外科接受切片檢查與拔牙，切片結果顯示此口腔内病灶爲持續進展之藍格罕細胞組織球增生症。建議病患繼續接治療並追加局部放射線治療，病人最後並未繼續於成大醫院完成治療且未持續就診。局部病灶之藍格罕細胞組織球增生症其預後良好，但多系統多病灶則超過半數會有持續性之疾病。此一病例報告爲藍格罕細胞組織球增生症，提出此一罕見系統性疾病與臨床病徵及治療概要以供進一步研究之參考。

並列關鍵字

藍格罕細胞組織球增生症 ； 口腔 ； 診斷 ； 治療