成人朗格汉斯细胞组织细胞增生症的临床病理学分析

Clinicopathologic analysis of Langerhans cell histiocytosis in adults

目的探讨成人朗格汉斯细胞组织细胞增生症（LCH）的临床特点、病理形态学、免疫组化特征及预后。方法回顾分析2005年1月至2011年12月宁波市临床病理诊断中心确诊的23例成人LCH患者的临床资料，病理组织学形态及免疫组化特征。结果23例LCH患者年龄20～58岁，平均37．2岁，男女之比为1．6：1。23例LCH有26处病变，其中骨组织14处（53．8％），其次为淋巴结、皮肤各4处（15．4％），软组织、肝脏、腮腺和颊部病变各1处（3．8％）。19例（82．6％）为单系统、单病灶，1例（4．3％）为单系统、多病灶，3例（13．0％）为多系统、多病灶。组织学上均有朗格汉斯细胞弥漫分布，背景中有不等量的嗜酸粒细胞、淋巴细胞、中性粒细胞及多核巨细胞。免疫组化结果显示朗格汉斯细胞CDla的表达率为100％（23／23），Langerin的表达率为100％（20／20），S-100蛋白和CD68的表达率分别为95．6％（22／23）和90．5％（19／21）。23例均行手术切除病灶或刮除病灶。16例获得随访资料，14例存活，其3年、5年总体生存率分别为92．9％和79．6％。结论成人LCH多发生在骨组织，以单系统、单病灶多见，治疗上以手术切除为主，预后较好。

Objective To explore the clinical features, pathomorphology, immunohistochemical characteristics and prognosis of Langerhans cell histiocytosis （ LCH ） in adults. Methods Twenty-three cases of adult LCH were retrieved from Ningbo Diagnostic Pathology Center during the period from January 2005 to December 2011. And their clinical presentation, pathomorphology and immunohistochemical characteristics were analyzed. Results The mean age of patients was 37.2 years （ range： 20 - 58 ）. The male-to-female ratio was 1.6： 1. Of 23 LCH patients, 26 lesions were found including 14 bone tissue lesions （53.8%） , followed by 4 lymph node lesions and 4 skin lesions （both 15.4% ） , as well as 1 soft tissue, liver, parotid and buccal lesion respectively （all 3.8% ）. Clinically, unisystem and unifocal disease was predominant （19 cases, 82. 6% ）, followed by unisystem and multifocal disease （1 case, 4. 3% ）, multi- system disease （3 cases, 13.0% ）. Histologically, all cases of LCH revealed diffused distribution of Langerhans cells, accompanied by a variable number of eosinophils, lymphocytes, neutrophils and muhinucleated giant cells. Immunohistochemically, the expression of CDla, Langerin, S-100 protein and CD68 was 100% （23/23）, 100% （20/20）, 95.6% （22/23） and 90. 5% （19/21） respectively. All lesions were treated by surgical therapy. Sixteen patients were available for follow-up examination and 14 patients survived. The 3 and 5-year cumulative survival rates were 92. 9% and 79. 6% respectively. Conclusions LCH of adults occurs predominantly in bone and presents mainly as unisystem or unifocal defects. Surgical excision is generally effective and the prognosis is fair.