160例成人朗格汉斯细胞组织细胞增生症患者临床特征分析  

A clinicopathological analysis of 160 cases of adult Langerhans cell histiocytosis

作　　者：许霞[1] 聂秀[2] 熊文[3] 潘华雄[2] 王威亚[1] 杨群培[1] 廖殿英[1] 刘卫平[1] 

机构地区：[1]四川大学华西医院病理科,成都610041 [2]华中科技大学同济医学院附属协和医院病理科 [3]武汉市普爱医院骨科

出　　处：《中华血液学杂志》2015年第2期135-139,共5页Chinese Journal of Hematology

基　　金：迈新病理基金（m1107）

摘　　要：目的 观察成人朗格汉斯细胞组织细胞增生症（LCH）患者的临床特征,以提高其早期诊断与治疗水平.方法 对1992年1月至2013年12月间年龄≥18岁的160例LCH患者资料进行回顾性分析.结果 160例患者中男110例,女50例（男、女比例为2.2∶1）,中位年龄33（18～73）岁.160例患者共有222个病变部位,包括骨骼172处（77.5％）,淋巴结13处（5.8％）,口腔8处（3.6％）,皮肤及肝脏各5处（2.2％）,眼眶及肺各4处（1.8％）,胸锁关节3处（1.4％）,消化道、耳及甲状腺各2处（0.9％）,肾上腺及舌下腺各1处（0.5％）.1个脏器受累150例（93.8％）,2个以上脏器受累10例（6.2％）.外院临床误诊77例（48.1％）,其中骨肿瘤（包括骨巨细胞瘤、纤维结构不良、软骨母细胞瘤、骨母细胞瘤及骨肉瘤）28例,骨结核13例,脑膜瘤9例,骨囊肿、慢性骨髓炎及尿崩症各5例,皮肤病及恶性淋巴瘤各4例,皮肤慢性溃疡、慢性中耳炎、肺癌及口腔癌各1例.结论 成人LCH患者以男性居多,以单系统病灶占绝对优势,多发生于骨骼系统.成人LCH临床表现多样化,易于延误诊断.Objectives To observe the clinical features of Langerhans cell histiocytosis （LCH）,and to improve its early diagnosis and treatment.Methods Retrospective analysis of 160 cases of adult LCH from pathology department,West China Hospital of Sichuan University and Union Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 1992 to December 2013 were performed,and their clinical features were analyzed.Results Of 160 cases,there were 110 male and 50 female,the male to female ratio was 2.2∶1.The mean age was 35 （18-73） years.There were total 222 lesion sites,including 172 （77.5%） osteal lesions,followed by 13 （5.8%） lymph nodes and 8 （3.6%） oral cavity lesions.The other involved organs were skin（5,2.2%）,liver（5,2.2%）,fossa orbitalis （4,1.8%）,lungs （4,1.8%）,sternoclavicular joint （3,1.4%）,gastrointestinal （2,0.9%）,ear （2,0.9%）,and thyroid （2,0.9%）,adrenal gland （1,0.5%） and sublingual gland （1,0.5%）.Of 160 cases,150 （93.8%） had one organ involved while 10 （6.2%） had two or more organs involved.Clinically,77 cases （48.1%） were misdiagnosed as bone tumors （28 cases,including giant cell tumor,fibrous dysplasia,chondroblastoma,osteoblastoma and osteosarcoma）,bone tuberculosis （13 cases）,meningioma （9 cases）,bone cysts （5 cases）,chronic osteomyelitis （5 cases） and diabetes insipidus （5 cases）,skin （4 cases） diseases malignant lymphoma （4 cases）,chronic skin ulcers （4 cases）,chronic otitis media （1 case）,lung （1 case） and oral cancer （1 case）.Conclusions In this group of the adult cases,the ratio of the male patients is higher.Adult LCH occurs predominantly in bone and presents mainly as unisystem single-focal disease,but multi-organ lesion and skin involvement are lower than that reported in the literatures.Just as LCH in children,adult LCH is also easy to be misdiagnosed.We should raise awareness of the disease and pathological examination is helpful for early di

关 键 词：组织细胞增多症 郎格尔汉斯细胞 成年人 误诊 临床特征 

分 类 号：R597[医药卫生—内科学]