구강악안면영역에서 발생한 랑게르한스 세포 조직구증 8례 - 증례보고 및 문헌고찰
Langerhans Cell Histiocytosis of the Oral and Maxillofacial Area: Report of 8 cases and Review of Literature
심혜원, 윤혜정, 홍성두, 이재일, 홍삼표,
소속 상세정보
심혜원 ( Shim Hye-Won ) - 서울대학교 치의학대학원 구강병리학교실
윤혜정 ( Yoon Hye-Jung ) - 서울대학교 치의학대학원 구강병리학교실
홍성두 ( Hong Seong-Doo ) - 서울대학교 치의학대학원 구강병리학교실
이재일 ( Lee Jae-Il ) - 서울대학교 치의학대학원 구강병리학교실
홍삼표 ( Hong Sam-Pyo ) - 서울대학교 치의학대학원 구강병리학교실
KMID : 0829220100340020095
Abstract
Langerhans cell histiocytosis (LCH) is a rare clinicopathologic disorder characterized by proliferation of histiocyte-like cells (langerhans cell histiocytes) accompanied by varying other inflammatory cells. LCH commonly involves the oral and maxillofacial region, but is very rarely seen. Then LCH has made it difficult to investigate the clinical and histological aspects. We investigated LCH of oral and maxillofacial region and analyzed clinical and histological characteristics. We reviewed the records of all patients who were diagnosed as LCH, retrospectively. Data included patient’s age, sex, chief complaint, clinical diagnoses, radiologic and histologic reports, and clinical course. We analyzed clinical and histological characteristics. From 2000 to 2007, 8 patients were diagnosed as LCH. 7 were children and 1 was adult. All cases involved mandible. Clinical type of all cases were “eosinophilic granuloma”. 6 cases were classified as “unifocal disease” and 2 cases were “multifocal single system diseases”. Microscopic findings commonly showed numerous histiocytes with eosinophilic cytoplasm (langerhans histiocytes). In 6 cases, immunohistochemical study was accomplished and confirmed the diagnosis of LCH. 6 cases were cured and not recurred, and 2 cases had loss of follow-up. Unifocal disease type of LCH may arise in Korean people more frequently than in western people (75% Vs 49%). Therefore, the higher frequency of unifocal disease of LCH is expected to raise the cure rate and to improve patient prognosis in Korean patients with LCH.
키워드
CD1a; CD207; Histiocytosis-X; Langerhans cell; Langerhans cell histiocytosis (LCH)
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