DeMarino D P, Dutcher P O, Parkins C W, Hengerer A S
Int J Pediatr Otorhinolaryngol. 1985 Oct;10(1):91-100. doi: 10.1016/s0165-5876(85)80062-8.
Histiocytosis X is a relatively rare disease presenting as 3 clinical syndromes: eosinophilic granuloma, Hand-Schuller-Christian disease, and Abt-Letterer-Siwe disease. Although the clinical expressions of the disease differ, the underlying histopathology appears to be the same. Since the disease frequently involves and not infrequently presents in the head and neck region, it is particularly relevant to the otolaryngologist. We have recently treated two young children presenting with bilateral otorrhea. Although histiocytosis X was entertained early in the evaluation of these patients, the diagnosis was difficult and prolonged in both cases. One case ultimately required electron microscopic study to confirm the diagnosis. Important aspects of the diagnosis are reviewed. Treatment of histiocytosis X includes surgery, radiation therapy and chemotherapy. These treatment strategies are discussed, with particular emphasis on the management of the ear involvement.
组织细胞增多症X是一种相对罕见的疾病，表现为3种临床综合征：嗜酸性肉芽肿、汉-许-克病和勒-雪病。尽管该疾病的临床表现各异，但其潜在的组织病理学表现似乎相同。由于该疾病常累及头颈部区域且不少见，因此对耳鼻喉科医生尤为重要。我们最近治疗了两名出现双侧耳漏的幼儿。尽管在对这些患者的评估早期就考虑到了组织细胞增多症X，但两例病例的诊断都很困难且耗时较长。其中一例最终需要进行电子显微镜研究以确诊。本文回顾了诊断的重要方面。组织细胞增多症X的治疗包括手术、放射治疗和化疗。本文讨论了这些治疗策略，特别强调了耳部受累的处理。
