郎格罕组织细胞增生症26例临床分析

Clinical analysis of 26 cases of langerhans cell histiocytosis

目的:郎格罕组织细胞增生症(Langerhans cell histiocytosis,LCH),临床表现复杂多样,容易误诊。本研究在于了解其临床特征,提高对该病的认识,减少误诊率。方法:回顾性分析本院2003年1月-2007年7月确诊的26例LCH患者的临床表现、影像、病理资料及治疗方法。结果:LCH侵犯的组织器官有骨骼20例(76.92%)、淋巴结2例(7.69%)、肺、脾、耳、肌肉各2例,肝、胃、垂体、鼻腔、口腔各1例;侵犯1个器官23例(88.46%),侵犯2个器官1例(3.85%),侵犯4个器官1例(3.85%),侵犯6个器官1例(3.85%)。临床表现多样化,症状与影像学缺乏特异性表现,确诊依靠病理诊断。单发病灶以手术、放疗为主。局部治疗效果好。结论:LCH临床表现多样,误诊率高。多系统病变采取化疗,单发病灶者手术及放疗疗效好。

Objective：To analyze Langerhalls cell histiovytosis （LCH） clinical features for the sake of improving diagnosis level and decreasing misdiagnosis rate. Methods ： Clinical data of 26 LCH patients was analyzed retraspectively. Results： The involved organs were bone （ 2 0/2 6,7 6.9 2 % ）, lymph nodes （ 2/2 6,7.6 9 % ）, lung （2/ 26,7.69% ）, spleen （2/26,7.69%）, ear （2/26,7.69%）, tousle （2/26,7.69%）, liver （ 1/26,3.85% ）, stomach （ 1/26,3.85% ） ,pituitary gland（ 1/26,3.85% ） ,nasal organ（ 1/26,3.85% ）, cavitas otis（ 1/26,3.85% ）. Of the 26 patients ,23 had 1 organ involved, 1 had 2 organs involved, 1 had 4 organs involved, 1 had 6 organs involved. The clinical manifestation of Langerhans cell histiocytosis （LCH） is diversified,but it is short of specificity . Definitive diagnosis of LCH is based on pathology. Surgical operation or radiotherapy for unifocal lesions may improve the prognosis. Condusion ： The clinical manifestation of LCH varies widely,leading to high rate of misdiagnosis. Combined chemo - therapy for multisystem lesions, surgical operation or radiotherapy for unifocal lesions may improve the prognosis.