郎格罕斯细胞组织细胞增多症临床特点和诊治进展  

Clinical characteristics and recent progress in the diagnosis and treatment for Langerhans cell histiocytosis

作　　者：刘跃平[1] 曲媛[1] 高黎[1] 刘新帆[1] 徐国镇[1] 李素艳[1] 蔡伟明[1] 肖建平[1] 李晔雄[1] 

机构地区：[1]中国医学科学院中国协和医科大学肿瘤研究所肿瘤医院放疗科,北京100021

出　　处：《中华放射肿瘤学杂志》2005年第3期185-188,共4页Chinese Journal of Radiation Oncology

摘　　要：目的　结合目前郎格罕斯细胞组织细胞增多症(LCH)研究进展,探讨LCH的临床特点、诊断、治疗、复发以及预后。方法　回顾性分析2 4年收治的LCH 5 5例,年龄2～6 7岁,男4 7例,女8例。单器官系统受侵4 0例,多器官系统受侵15例。治疗以手术切除和放疗为主,手术为部分或全部切除病灶,放疗为受累野照射(中位剂量30Gy)。结果　LCH以头颈部软组织受侵多见(占6 3.6 % ) ,骨次之(占2 3.6 % )。骨受侵率在≤15岁组和>15岁组分别为6 6 .7%和11.6 % (P <0 .0 1)。5、10年总生存率均为10 0 % ,无病生存率分别为70 .9%、5 8.4 %。5年无病生存率在≤15岁组和>15岁组分别为5 8.3%和74 .4 % (P =0 .830 ) ,单器官系统受侵组和多器官系统受侵组分别为75 .0 %和6 0 .0 % (P=0 .130 )。总复发率为4 3.6 % ,2 / 3在5年内复发,其中新病灶占75 %。结论　LCH并非儿童特发;头颈部为LCH好发部位;其骨受侵率与年龄相关;复发率较高;Objective To discuss the clinical characteristics, reactivation, d iagnosis, treatment and prognosis of Langerhans cell histiocytosis (LCH) based o n the new progress. Methods Fifty-five such patients treated from Jan. 1974 t o May 1998 were retrospectively analyzed. The patients aged from 2 to 67 years at the time of diagnosis, with a median age of 31 years,male 47, female 8. Forty patients had single system disease and the other 15 with multisystem disease. P artial or total excision and/or local radiotherapy were the main treatments. The involved irradiation field was given, with a median dose of 30?Gy. Results The soft tissue of head and neck was the most frequently involved (63.6%), followed by the bone (23.6%). Patients ≤ 15 years had more bony involvement (66.7%) comp a red to those aged >15 years (11.6%) (P<0.01). At a median follo w-up of 12 years , no patient had died from LCH. The probability of overall 5- and 10-year surv iv al were 100%, disease-free survival were 70.9% and 58.4%, respectively. The 5- ye ar disease-free survival estimate for patients aged ≤ 15 years and > 15 years we re 58.3% and 74.4%, respectively, (P=0.830) ;whereas it was 75. 0% for single sy stem disease and 60.0% for multisystem disease (P=0.130). The to tal reactivation for all patients was 43.6%. Two thirds of these patients developed recurren ce w ithin 5 years after treatment with 75% developing new foci. At the time of the l ast follow-up, 74.5% of the patients have survived without disease, while 27.6% o f survivors still had active disease. Conclusions Langerhans cell histiocyto sis, not being confined to children, involves the head and neck area most common ly. The frequency of bony invasion is associated with age. Reactivation is very frequent. Patients without risk organs involvement usually carry a favorable pro gnosis.

关 键 词：组织细胞增多症 郎格罕斯细胞 临床特点 诊治进展 无病生存率 器官系统 头颈部软组织 回顾性分析 LCH 手术切除 全部切除 总生存率 好发部位 年龄相关 危险器官 复发率 5岁 组分 治疗 预后 放疗 病灶 

分 类 号：R55[医药卫生—血液循环系统疾病]