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成人朗汉斯细胞组织细胞增生症 被引量:4

Adult Langerhans Cell Histiocytosis
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摘要 目的:探讨我国成人朗汉斯细胞组织细胞增生症(LCH)的临床分型、分级及治疗情况。方法:对我院近10年来收治的成人LCH9例和1995~2005年国内文献中报告的成人LCH286例作一综合分析。结果:成人LCH295例,男207例,女88例,男女之比为2.4:1,年龄18~75岁,平均35.0岁。骨为最多受侵犯(81.4%),其次为淋巴结、肺和皮肤。按传统分型,骨嗜酸肉芽肿(EGB)最多见(75.9%),其次为单一器官型(13.9%)。按国际组织细胞协会分型,绝大多数为单系统型(96.3%),其中单系统单部位型63.4%,单系统多部位型32.9%。结论:成人LCH发病隐袭,常为慢性病程,多为侵犯骨骼的单系统疾病。原发性肺受累是成人LCH的又一特征,成人LCH总体预后良好。 Objective:To explore clinical classification, grade, and treatment of adult Langerhans cell histiocytosis(LCH) in China. Method: 9 adults with LCH treated in Fuzhou General Hospital from 1995 to 2005 and 286 a dults with LCH reported in the literature in China were analyzed. Result: The age of onset ranged from 18 to 75 years in 207 males and 88 females with average of 35 years old. Bones were mostly damaged (81.4%), lymph nodes, lungs, skin were less damaged. According to the traditional classification, most (75.9 % ) belonged to Eosinophilic granuloma of bone (EGB). 13.9% belonged to single-organ. According to the Lavin-Osband criteria, most (92.5%) belonged to grade Ⅰ. According to the International Histiocyte Association, most (96.3%) belonged to single-system. Conclusion: Adult LCH is a chronic disease. Most cases belonge to single-system with bone injury. Primary lung injury is the other characteristic of adult LCH. On the whole, the prognosis of adult LCH is well.
作者 吴颖 陈捷
出处 《临床血液学杂志》 CAS 2006年第5期284-286,共3页 Journal of Clinical Hematology
关键词 组织细胞增多症 朗汉斯细胞 成人 疾病严重程度指数 Histiocytosis, langerhans cell Adult Severity of illness index
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