成人口腔多灶性朗格汉斯细胞组织细胞增生症1例

Multifocal oral Langerhans cell histiocytosis in adult:A case report

朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是一种朗格汉斯细胞异常增殖和浸润性疾病,通常累及全身骨骼、皮肤、垂体,亦可累及肝脏、脾、血液系统、肺、淋巴结及中枢神经系统。成年人罕见。LCH缺乏公认的治疗方案,最常见的治疗方法包括手术、局部注射类固醇药物、化疗、放疗或联合治疗。本病案报道1例采用局部小剂量放射治疗的成年男性上下颌骨多灶性LCH,结合该病例的诊治经验及既往文献报道,讨论了口腔单系统LCH合并多灶性骨病变的治疗方案选择。

Langerhans cell histiocytosis(LCH),an abnormal proliferative and infiltrative disorder of Langerhans cells,usually involves skeleton,skin,pituitary and can also affect liver,spleen,blood system,lungs,lymph nodes,and central nervous system.LCH is rare in adults.It lacks a recognized therapeutic regimen,and therapies of LCH mainly include surgery,topical injections of steroids,chemotherapy,radiotherapy,and combined therapy.Herein,we report an adult patient with oral single system(SS)-LCH with multifocal bone lesions involving the maxilla and mandible treated with local small-dose radiotherapy.We discuss the therapeutic regimen selection of oral single system LCH combined with multifocal bone lesions,with the help of its diagnosis,treatment experience and previous literature reports.