骨朗格汉斯细胞组织细胞增生症22例临床病理分析  

Langerhans cell histiocytosis of bone:a clinicopathological analysis

作　　者：钱利华[1] 鲍冬梅[1] 郑红芳[1] 曲华毅[2] 虞有智[1] 沈丹华[1] 

机构地区：[1]北京大学人民医院病理科,北京100044 [2]北京大学人民医院骨肿瘤科,北京100044

出　　处：《诊断病理学杂志》2007年第4期267-270,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的 探讨骨朗格汉斯细胞组织细胞增生症（LCH）的临床病理和免疫组化特征，分析需要进行鉴别诊断的疾病。方法 收集22例组织学证实为骨LCH的病例，分析总结其临床及病理学特征，并进行免疫组化染色观察。结果 本组患者年龄2～37岁，儿童比成人多见。任何部位的骨组织均可受累。临床以局部疼痛及肿胀为主要表现，X线表现不一。组织病理学检查示受累骨组织可见形态特殊的组织细胞，即朗格汉斯细胞组织细胞呈不同程度增生，同时伴有数量不等的嗜酸细胞、淋巴细胞、中性粒细胞及破骨样巨细胞。免疫组化染色CD1a和S-100（＋），部分CD68（＋）。结论 骨LCH的病因尚未明确，其组织病理学上具有特征性表现。需要与骨髓炎、骨肿瘤以及其他组织细胞增生性疾病鉴别，免疫组化染色CD1a、S-100和CD68等标记对确诊有帮助。多数孤立性骨LCH患者预后良好，但可有少数病例复发。Objective To investigate the clinicopathological, immunohistochemical characteristics and differential diagnosis of Langerhans cell hisfiocytosis （LCH） of the bone. Methods Twenty-two patients with histologically proven LCH in the bone were collected and their clinical and pathological features were reviewed, Immunohistochemical finding was studied. Results The age of the patients with bone LCH in this group ranged from 2 to 37 years. It occured more frequently in children than in adults. LCH could involve any bone. The most common presenting complaint was pain and tissue swelling, The radiographs revealed heterogenous features. Pathological examinations showed the proliferation and accumulation of a specific histiocyte： the Langerhans＇ cell, admixed with variable numbers of eosinophils, lymphocyte, neutrophils and some. osteoclast-like multinucleated giant cell （MGC）. Immunohistochemically, Langerhans＇ cells were strongly positive for CDla, S-100 protein and occasionally positive for CD68. Conclusions Bone LCH is a disorder of tmknown cause and with distinctive morphologic findings. It needs to be distinguished from osteomyelitis, neoplasms of bone, and other histiocytosis diseases. Immunohistochemical staining for CD1a, S- 100 protein and CD68 is helpful in confirming the diagnosis. In most patients with isolated bone LCH, the prognosis is excellent. The recurrences are less frequent.

关 键 词：朗格汉斯细胞组织细胞增生症 骨 嗜酸性肉芽肿 免疫组化 

分 类 号：R733.1[医药卫生—肿瘤]