- 期刊
Langerhans Cell Histiocytosis in Taiwan: A Retrospective Case Series in a Medical Center
蘭格罕氏細胞組織細胞增生病：－醫學中心之回溯性研究
摘要
背景：蘭格罕氏細胞組織細胞增生病是一群由蘭格罕氏細胞在不同器官的浸潤所造成的多樣化的疾病。 目的：描述該疾病在台灣人種的表現，及進一步探討與西方人種病徵有無差異。 方法：我們回溯研究台灣一家醫學中心1990年與2006年間，被診斷為蘭格罕氏細胞組織球增生症的病人。 結果：本研究一共蒐集了35個經病理診斷證實的病例，年齡中位數為3歲，其中5位診斷時超過18歲。男女比為3比2。其中15位(42.8%)為多系統侵犯，12位(34.2%)為骨骼系統單一侵犯，3位為皮膚侵犯，其餘5位各為淋巴節、肺臟、甲狀腺、下頷唾腺及眼外軟組織的單一侵犯。多系統侵犯的病人中，10位有皮膚或黏膜病灶，10位有骨骼侵犯。一共有三例死亡，皆為多系統侵犯之病人。 結論：與西方文獻相比，我們發現本研究的病人以單一肺臟侵犯為表現的病例明顯較少(2.8%)，此發現還需要日後跨醫學中心、前瞻性的研究來進一步證實。
關鍵字
無資料並列摘要
Background: Langerhans cell histiocytosis (LCH) comprises a diverse group of disorders where pathologic Langerhans cells accumulate in a variety of organs. Objectives: To characterize the disease presentation in Taiwanese, and to analyze the differences of LCH between Taiwanese and western populations. Methods: A retrospective chart review was performed on patients diagnosed with LCH in a medical center in Taiwan between 1990 and 2006. Results: Thirty-five patients with biopsy-proven LCH were included. The median age of diagnosis was 3 years (range, 2 months to 58 years), and five patients aged 18 or older at the time of diagnosis. The male: female ratio was 3: 2. Multisystemic involvement was found in 15 patients (42.8%) and isolated bone lesions were found in 12 patients (34.2%), 3 patients had isolated cutaneous involvement; the remaining 5 patients had solitary lymph node, lung, thyroid gland, submandibular gland or extraocular soft tissue involvement, respectively. Ten of the 15 patients with multisystemic diseases had skinlmucosal involvement; 10 had bony involvement. All three patients that expired had multisystemic disease. Conclusion: Compared to western literature, we found significantly less isolated lung involvement (2.8%) in our cohort. Further multi-centered, prospective researches may be needed to better characterize the disease presentation in Asian population.
延伸閱讀
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