Salam Hira, Shahid Ruqaiya, Mirza Talat
Department of Oral Pathology, Dr. Ishratul Ibad Khan Institute of Oral Health Sciences, Dow University of Health Sciences, Ojha Campus, Karachi.
Department of Pathology, Dow International Medical College, Dow University of Health Sciences, Ojha Campus, Karachi.
J Coll Physicians Surg Pak. 2017 Sep;27(9):S89-S91.
Langerhans cell histiocytosis (LCH) is the latest terminology for a disorder of reticulo-endothelial system, previously known as histiocytosis X, and marked by aberrant proliferation of bone marrow derived Langerhans cells with variable inflammatory infiltrate including neutrophils, lymphocytes, plasma cells, eosinophils, and multinucleated giant cells. Although rare, the disorder frequently inflicts children with peak incidence recorded in 2-4 years age group. LCH is rare in adults. A22-year adult male presented with the complaint of teeth mobility. Orthopantomogram (OPG) revealed multiple extensive osteolytic lesions destroying both jaws. Giant cell granuloma, Paget's disease and LCH, along with other vanishing bone disorders (Gorham Stout disease and Winchester syndrome) were suspected differentials. Multiple incisional biopsies were performed and a diagnosis of LCH was confirmed by routine histopathological analysis followed by immunohistochemical staining for S-100 and CD1a.
朗格汉斯细胞组织细胞增多症（LCH）是网状内皮系统疾病的最新术语，以前称为组织细胞增多症X，其特征是骨髓来源的朗格汉斯细胞异常增殖，并伴有包括中性粒细胞、淋巴细胞、浆细胞、嗜酸性粒细胞和多核巨细胞在内的不同程度的炎症浸润。尽管这种疾病罕见，但它经常影响儿童，发病高峰年龄在2至4岁组。LCH在成人中罕见。一名22岁成年男性因牙齿松动前来就诊。全景曲面断层片（OPG）显示双侧颌骨有多个广泛的溶骨性病变。巨细胞肉芽肿、佩吉特病和LCH，以及其他骨质消失性疾病（戈勒姆-斯托特病和温彻斯特综合征）被怀疑为鉴别诊断。进行了多次切开活检，通过常规组织病理学分析并随后进行S-100和CD1a免疫组化染色确诊为LCH。
