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.
出处
《临床血液学杂志》
CAS
2006年第5期284-286,共3页
Journal of Clinical Hematology