組織細胞增生症之臨床經驗

Clinical Experience of Histiocytosis X

吳家淦(Stephen Wu) ； 何青吟(Ching-Yn Her) ； 蕭安穗(An-Suey Shiao)

《中華民國耳鼻喉科醫學會雜誌》 30卷2期 (1995/04) Pp. 145-149

https://doi.org/10.6286/1995.30.2.145

摘要

組織細胞增生症(histiocytosis X)是一種罕見的疾病，臨床上患者常有頭頸部的症狀，所以耳鼻喉科醫師與這種疾病的關係十分密切。從1981年至1992年我們共診斷了14個組織細胞增生症病例，患者多為5歲以內的男孩；85.7%的病患於病程中出現頭頸部的症狀，其中以頭顱的突出性腫塊最多，其它依次是眼球突出、頸部腫塊、耳漏、皮膚疹、口腔黏膜的腫塊、以及突發性失明等。尿崩症的發生率為21.4%。
胸部X光檢查、顱骨常規攝影、電腦斷層攝影、腹部超音波、和全身骨骼核子掃描等能夠幫忙找出病灶的位置與數目，確定診斷則須借助於組織病理的分析。根據病灶的部位和嚴重程度，這些患者分別接受手術、放射線、化學藥物、或混合性的治療。經過平均4年的追蹤，35.7%的患者獲得痊癒，35.7%的患者進入慢性期，而死亡率為28.6%。

關鍵字

組織細胞增生症 ； 頭頸部的症狀

並列摘要

Histiocytosis X (H-X) is a rare disease, characterized by the proliferation of histiocytes in various tissues and organs. But it is relevant to otolaryngologists because patients with this malady present with, or ultimately develop, head and neck manifestations. Fourteen patients with histologically confirmed H-X have been treated at Taipei Veterans General Hospital from 1981 to 1992.
Overall, during the course of the disease, 85.7% of the patients developed head and neck manifestations. Lesions of the skull occurred most frequently, followed by exophthalmos, neck mass cephalic rash, mass of the buccal mucosa and sudden onset blindness.
The diagnosis of H-X is confirmed by biopsy and histopathological study of the suspected lesions. The treatment must be appropriate for the extent and severity of the disease. Singualr bony lesions of eosinophilic granuloma are treated with surgical curettage and / or localized radiation of 600 to 1000 rads. Multifocal H-X is usually treated with chemotherapy or combined therapy. Disseminated H-X remains a frequently fatal disease, despite systemic chemotherapy. After a 2- to 10- year follow-up, 35.7% of patients were alive with no evidence of active disease and 35.7% of patients entered chronic phase. The mortality rate was 28.6%.

並列關鍵字

histiocytosis X ； head and neck manifestation