瀰漫性Langerhans氏細胞組織球症

Disseminated Langerhans' Cell Histiocytosis

游進益(Ching-Yi Yiu) ； 徐惠珍(Hui-Chen Hsu) ； 林永松(Yung-Song Lin) ； 曾慶誠(Ching-Cherng Tzeng)

《台灣耳鼻喉頭頸外科雜誌》 38卷s_1期 (2003/01) Pp. 48-53

https://doi.org/10.6286/2003.38.s_1.48

摘要

Langethans氏細胞組織球症是一種相當罕見的疾病，發生率約為2百萬分之一，主要發生於小孩或年輕人。臨床症狀具有多樣性，可以由單一或多發骨頭侵犯至多種器官侵犯；其治療原則也隨著不同的臨床表現而有不同的治療方法。奇美醫學中心耳鼻喉部於2001年12月經歷1例15個月大的女童，主要症狀為左側臉頰腫脹約有2星期，經電腦斷層及核磁共振檢查診斷為左側下頜骨腫瘤，病人接受手術切除，病理診斷為嗜伊紅性肉芽腫。經3個月追蹤出現多發性骨骼及肺部侵犯，進展為Letterer-Siwe病，屬於急性多發性Langethans氏細胞組織球症，之後接受化學治療，初步治療的效果良好。因此種病例相當罕見，特提出報告。

關鍵字

Langerhans氏細胞組織球症 ； 嗜伊紅性肉芽腫

並列摘要

Langerhans' cell histiocytosis, previously known as histiocytosis X, is a rare disease with incidence about 1 in 2 million. It often affects children and young adults, without significant sex predilection. Although the pathogenesis is still indistinct, Langerhans' cells originate from either local proliferation or dissemination of dendritic cells of the bone marrow. The broad clinical spectrum of the disease ranges from solitary bone lesion to various degrees of other organ involvement. The former can be cured by simple curettage and the later may present with significant mortality. However, there are no histologic differences in these various disease spectrums. We experienced a case of disseminated Langerhans' cell histiocytosis with initial presentations of tender swelling of left cheek in a 15-month-old girl. The CT scan and MRT showed a 2×3 cm cystic lesion with soft tissue expansion in the ascending ramus and angle of the left mandible. Wide excision of the tumor was performed, which was pathologically proved to be an eosinophilic granuloma. Unfortunately, multiple bony and pulmonary involvement were noted 3 months later postoperatively. Thus, clinical courses progressed to disseminated Langerhans' cell histiocytosis. She responded well to chemotherapy.

並列關鍵字

Langerhans' cell histiocytosis ； eosinophilic ganuloma