儿童眼眶朗格汉斯细胞组织细胞增生症的临床特征及治疗分析  

Clinical characteristics and treatment of children orbital Langerhans cell histiocytosis

作　　者：张少波 王萍 程启琳 张琼 宁小娜 李养军 Zhang Shaobo;Wang Ping;Cheng Qilin;Zhang Qiong;Ning Xiaona;Li Yangjun(Department of Ophthalmology,the Second Affiliated Hospital,Air Force Military Medical University,Xi’an 710038,China)

机构地区：[1]空军军医大学第二附属医院眼科,西安710038

出　　处：《眼科》2021年第4期300-306,共7页Ophthalmology in China

摘　　要：目的探讨儿童眼眶朗格汉斯细胞组织细胞增生症(LCH)的临床特征及治疗方案。设计回顾性病例系列。研究对象2013年4月-2019年7月空军军医大学第二附属医院经病理确诊的眼眶LCH患者7例(8眼)。方法整理、分析患者一般情况、临床表现、影像学资料、病理特征、治疗方案及随访结果。主要指标临床表现,病理特征、治疗方案和随访结果。结果7例中5例为单灶性病变,2例为多灶性病变,均为男性,年龄8个月~10岁。病程3天~2个月。单灶性病变者,单眼发病,以眼睑肿胀为主要表现;多灶性病变者,单眼和双眼均可发病,以眼球突出为主要表现。眼眶CT:单灶性病变:眼眶外侧及外上方软组织肿物伴周边颅眶骨的典型虫蚀样破坏。多灶性病变:单眼或双眼眶软组织肿物伴颅眶骨多发典型的地图样骨缺损,1例伴肋骨虫蚀样破坏。眼眶MRI:肿物在T1WI呈中低信号,T2WI中高混杂信号,T1增强后病灶边缘及周围组织明显强化,病灶中心不均匀明显强化。组织形态学:病理性郎格汉斯细胞的克隆性增生,伴单核细胞、淋巴细胞、嗜酸细胞等浸润;免疫组化染色:CD1a、CD207和S100呈阳性信号。单灶性病变行手术完全切除+骨腔刮除+骨质缺损修复+术中/术后局部曲安奈德注射。多灶性病变1例单眼受累者行手术完全切除+刮除骨腔+局部曲安奈德注射,1例双眼受累者行手术活检。术后均行全身静脉化疗。单灶性病变随访30~85个月,病变均完全消失,无复发;多灶性病变随访12~13个月,病变无进展。结论儿童眼眶LCH典型表现为眼睑肿胀或眼球突出,CT或MRI影像学及病理学检查是主要诊断依据。单灶性病变可行手术切除病灶、刮除骨腔联合局部糖皮质激素注射;多灶性病变需排除其他系统疾病,术后需全身静脉化疗控制病情进展。(眼科,2021,30:300-306)。Objective To investigate the clinical features and treatment of children orbital Langerhans cell histiocytosis(LCH).Design Retrospective case series.Participants From April 2013 to May 2019,7 patients(8 eyes)with orbital LCH were confirmed in the Second Affiliated Hospital of Air Force Military Medical University.Method The general situation,clinical manifestations,imaging and general examination,pathological features,treatment plan and clinical follow-up results were summarized.Main Outcome Measures Clinical and pathological characteristics,treatment and follow-up results.Results Of the all 7 patients,5 were unifocal lesions and 2 were multifocal lesions.All patients were male with an age range of 8 months to 10 years.Course range was 3 days to 2 months.The clinical manifestations of unifocal lesions were monocular,with eyelid swelling as the main symptom,1 case with eyelid mass.The clinical manifestations of multifocal lesions were monocular or binocular eyes,with exophthalmos as the main symptom,and 1 case with red eyes and tears.On CT images in unifocal lesions,there were lateral and upper orbital soft tissue mass with typical wormlike destruction of peripheral cranio-orbital bone.In multifocal lesions,there was monocular or bilateral orbital soft tissue mass with multiple map bone defect of cranio-orbital bone,and 1 case with insect-like destruction of rib.There were iso-hypointense on T1-weighted MR imaging and iso-hyperintense mixed signals on T2-weighted imaging.With T1 enhancement,most of the cases lesions presented obvious marked enhancement at the edges and the surrounding tissues,and with heterogeneous obvious enhancement of the lesion center.On histopathology,there were clonal proliferation of pathological Langerhans cells with infiltration of monocytes,lymphocytes and eosinophils.On immunohistochemical staining,CD1a,CD207 and S100 were showed positive signals.In unifocal lesions,all the 5 cases were treated with complete resection,curettage of bone cavity and repair of bone defect,combined with intr

关 键 词：眼眶朗格汉斯细胞组织细胞增生症/临床表现 眼眶朗格汉斯细胞组织细胞增生症/治疗 

分 类 号：R777.5[医药卫生—眼科]